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Autosomal dominant transmission of the 'photic sneeze reflex'
Abstract
Sneezing in response to bright light is a common yet poorly documented phenomenon. In the only major epidemiologic study ever performed, Everett called this entity the 'photic sneeze reflex' and found it to be present in 23 per cent of the medical students at Johns Hopkins. We now report a documented family history of the photic sneeze reflex. In the index patient, movement from an indoor location to bright sunlight invariably results in two sneezes. This phenomenon is also present in the father and brother of the index patient but is absent in his mother and wife.
... Interestingly, a higher incidence in people with a high intelligence [7] and a significant higher incidence in children aging 4 weeks to 33 months were observed [2,15]. Moreover, Collie et al. [6] described a family history of this extraordinary reflex, so the authors suggested an autosomal dominant inheritance and named this reflex ''Autosomal Cholinergic Helio Ophtalmologic Outburst''. ...
... But this question can be answered only by an appropriate test procedure. Nevertheless, the already suspected familial aggregation [15] was confirmed by us and the suspicion of a genetic component could become corroborated. Unfortunately, a more detailed analysis of the inheritance was not possible because of the layout of our study. ...
... As already mentioned, our study showed that the photoinduced sneeze reflex appears to be an underestimated phenomenon as the prevalence is significantly higher than previously described in the literature [2,11,15], making a corresponding potential impact of this stimulus conceivable to everyday situations. ...
The sneezing reflex is a common phenomenon in human beings and animals. Until now largely uninvestigated sneezing phenomenon is the photic sneeze reflex, also known as the ACHOO (Autosomal Cholinergic Helio-Ophtalmologic Outburst). This light induced sneezing reflex is triggered by a bright light like the sun but also by artificial light. In former publications the prevalence of this phenomenon in the population is specified up to 35 % and can result in a potential endangerment in numerous everyday situations as driving a car or controlling an airplane. In our present study we interviewed 1042 patients in the Department of Otorhinolaryngology of the Saarland University Hospital using standardized questionnaires to analyse epidemiological data as age, gender and known diseases. Additionally, the questionnaire focused on the occurrence of sneezing events, extend of sneezing, potential triggers, and methods of evasion. In our data analysis, we were able to show a high prevalence of the ACHOO in 57 % of our cohort, suffering from regular light induced sneezing events. Excluding subjects reporting only occasional sneezing events our results were comparable to findings in literature. As expected a familial disposition was observed, additionally tobacco smoking correlated with light induced sneezing events. Analyzing a large cohort, we were able to demonstrate that the prevalence of the ACHOO in the german population may be higher than expected. Taken into account the potential hazard caused by this phenomenon, further investigations should identify the pathophysiologic pathway.
... Autosomal Dominant Compelling Helio-ophthalmic Outburst Syndrome (ACHOO) is very widespread, but few published reports discuss the pathophysiology of the syndrome. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] The first reference is attributed to Aristotle. 18,24 It consists of a variable amount of sneezing in uncontrollable outbursts after sudden exposure to bright light, usually sunlight. ...
... 18,24 It consists of a variable amount of sneezing in uncontrollable outbursts after sudden exposure to bright light, usually sunlight. [1][2][3][4][5][6][7][8][9][10][11][12][13]15,16 Inheritance is autosomal dominant, 1,2,[8][9][10][11][12][13]18 with high penetrance, without preference for gender or iris colour, 8,11,12 but it is more common in Caucasians. 11,15 Prevalence ranges from 11% to 35%. ...
... 18,24 It consists of a variable amount of sneezing in uncontrollable outbursts after sudden exposure to bright light, usually sunlight. [1][2][3][4][5][6][7][8][9][10][11][12][13]15,16 Inheritance is autosomal dominant, 1,2,[8][9][10][11][12][13]18 with high penetrance, without preference for gender or iris colour, 8,11,12 but it is more common in Caucasians. 11,15 Prevalence ranges from 11% to 35%. ...
Objective:
To assess ocular involvement in the pathophysiology of autosomal dominant compelling helio-ophthalmic outburst syndrome (ACHOOs).
Methods:
An interview was conducted with a Caucasian family that showed clinical features of ACHOOs. Twelve of them had photic reflex and were recruited. A complete eye evaluation was made.
Results:
A dominant autosomal inheritance with mild penetrance was demonstrated, with 67% of the studied subjects showing some degree of prominent corneal nerves. No other eye changes were found.
Conclusions:
Prominent corneal nerves may be associated with ACHOOs. The other eye structures studied do not seem to play a role in ACHOOs. Further studies are needed to understand the physiology of the ACHOOs.
... Estudiamos una familia española que presentaba reflejo fótico construyendo el árbol genealógico para verificar el número de pacientes afectos y el patrón de transmisión ( fig. 1). Nuestro caso, como el publicado por Peroutka y Peroutka 10 , es el de la familia del propio autor (C. Sevillano). ...
... El ACHOOs se caracteriza por salvas de estornudos paroxísticos, tras la exposición repentina, de un sujeto adaptado a la oscuridad, a una luz brillante 1,15,16 , manifestándose habitualmente desde la infancia 2,9,10 . Hay 4 teorías principales que explican este fenómeno 1,2,4,7,9,[11][12][13]18 . ...
To assess ocular involvement in the pathophysiology of autosomal dominant compelling helio-ophthalmic outburst syndrome (ACHOOs).
... This type of study would involve a non-health risk condition such as the autosomal dominant compelling helio-ophthalmic outburst (ACHOO) syndrome [13]. This syndrome is found in roughly 30% of the population and involves sneezing when initially exposed to bright light, also known as a photic sneeze reflex [14]. ...
... Since not all biallelic markers generate informative data, as shown in this study, this advancement increases the possibility of identifying informative markers for students to determine Lod scores in order to assess linkage of the marker to the trait. In this case, mapping of the gene for the ACHOO syndrome was chosen since roughly 30% of the population have this non-health risk trait [14], and therefore identifying families to participate would be relatively easy. Since the determination of Lod scores is additive, then a proposed database can be generated for different laboratory courses to incorporate their data for the 12 different markers until significance for linkage or non-linkage is reached, hence students are participating and contributing real data in an ACHOO syndrome mapping consortium. ...
As DNA profiling systems become more complex, advancements to a relatively simple technique are presented that promote greater accessibility and usefulness for a variety of applications. In contrast, other simple tools commonly used to teach students about forensics and human populations have notable drawbacks. Two Alutetraplex systems, utilizing four Alu presence/absence variants in a single reaction were therefore developed to provide a simple methodology to generate complex profiles. A third Alutetraplex system is presented, escalating the number of possible genotypes to 531,441, with all alleles of the 12 dimorphic markers being relatively common. Reproducible results were attained even with the use of crude DNA preparations stored frozen for several years with multiple freeze thaws. The incorporation of GelRed DNA stain instead of the highly toxic ethidium bromide promotes greater accessibility, particularly in a classroom setting. This study demonstrates the effectiveness of this profiling system as a simple but informative methodology to analyze paternity, genetic mapping of human traits, and provides data to illustrate its potential in assessing ancestry or geographic origins of an individual.
... Sneezing in response to bright light is a common but poorly understood phenomenon, in which an estimated 25% of people sneeze when exposed to a very bright light stimulus, such as stepping outside of a building into bright daylight. In the scientific literature and common usage, this phenomenon has been termed the photic sneeze reflex (PSR) (Anderson and Rosenblith 1968;Leavitt 1984;Peroutka and Peroutka 1984;Whitman and Packer 1993;Keeton 1995), the solar sneeze reflex (Lang and Howland 1987), the photic sneeze syndrome (PSS) (Sasayama et al. 2019;2018), autosomal dominant compulsive helio-ophthalmic outbursts of sneezing (ACHOO) (Subramanian and Shetty 2023;Collie et al. 1978;Morris 1987;1989;Elwood et al. 1996;McKusick 2003;Dean 2012), photic sneezing or simply sun sneezing Communicated by Melvyn A. Goodale. ...
The photic sneeze reflex (PSR) is an involuntary sneezing response to bright light exposure, affecting approximately 25% of the population. Despite its long history in scientific literature, the underlying mechanisms remain unclear. Several theories, including optic-trigeminal summation, parasympathetic hypersensitivity, and parasympathetic generalization, have been proposed, but none have been conclusively validated. Reproducing the PSR reliably in a laboratory setting is crucial for understanding its neural underpinnings, yet the specific light parameters that trigger PSR are not well-defined. This mini-review aims to consolidate current knowledge on the light stimulus parameters (intensity, spectral composition, wavelength, duration, timing, spatial configuration) that elicit the PSR. A comprehensive literature search was conducted using MEDLINE (PubMed), Google Scholar, Google Books, and Google, employing terms related to photic sneezing in multiple languages. Articles from 2020 to 2024 were screened, resulting in 167 records, with seven studies focusing on stimulus parameters included in this review. The reviewed studies, including four case reports and three laboratory experiments, consistently support that bright light can induce sneezing in susceptible individuals. However, there is significant variability in the methodologies and outcomes, limiting comparability and indicating a need for systematic investigation. No study has yet examined the parametric relationship between light parameters and the PSR. The heterogeneity of methods and findings in the existing literature highlights the lack of standardized research on the specific light parameters that trigger the PSR. This review underscores the need for controlled experiments to clarify these relationships and improve our understanding of the underlying neural mechanisms. Existing research on photic sneezing stimulus parameters is fragmented and lacks systematic approaches. Future studies should focus on standardized, parametric investigations to elucidate the light-sensitive mechanisms of the PSR.
... Taken together, the data from these early studies suggested that photic sneezing is much more common in Caucasians than in blacks, with a slight male preponderance. The inheritance appeared to be Mendelian autosomal dominant, as reflected by the A in the name ACHOO [Collie et al., 1978;Peroutka & Peroutka, 1984;Morris, 1987]. A 2010 study demonstrated a correlation between photic sneeze reflex (PSR) and a single-nucleotide polymorphism on chromosome 2 [Eriksson et al., 2010]. ...
... With the two genetic markers available, the biology underlying the PSR phenotype remained mysterious, and even the previous report of autosomal dominant inheritance was not conclusive 28 . A particularly interesting observation was that the variant of rs1032507 is adjacently upstream of the coding region of CADM2. ...
Photic sneeze reflex (PSR) is an interesting but yet mysterious phenotype featured by individuals’ response of sneezing in exposure to bright light. To uncover the underlying genetic markers (single nucleotide polymorphisms, SNPs), a genome-wide association study (GWAS) was conducted exclusively in a Chinese population of 3417 individuals (PSR prevalence at 25.6%), and reproducibly identified both a replicative rs10427255 on 2q22.3 and a novel locus of rs1032507 on 3p12.1 in various effect models (additive, as well as dominant and recessive). Minor alleles respectively contributed to increased or reduced risk for PSR with odds ratio (95% confidence interval) at 1.68 ([1.50, 1.88]) for rs10427255 and 0.65 ([0.58, 0.72]) for rs1032507. The two independent SNPs were intergenic, and collectively enhanced PSR classification by lifting the area-under-curve value in ROC curve to 0.657. Together with previous GWAS in other populations, the result substantiated the polygenic and non-ethnicity-specific nature behind the PSR phenotype.
... Photic sneeze. There is evidence that photic sneeze reflex is genetic: Peroutka and Peroutka [38] concluded that this trait is inherited in an autosomal dominant fashion. We find one region associated with photic sneeze reflex about 725kb away from ZEB2 and a second suggestively associated about 550kb away from NR2F2. ...
Despite the recent rapid growth in genome-wide data, much of human variation remains entirely unexplained. A significant challenge in the pursuit of the genetic basis for variation in common human traits is the efficient, coordinated collection of genotype and phenotype data. We have developed a novel research framework that facilitates the parallel study of a wide assortment of traits within a single cohort. The approach takes advantage of the interactivity of the Web both to gather data and to present genetic information to research participants, while taking care to correct for the population structure inherent to this study design. Here we report initial results from a participant-driven study of 22 traits. Replications of associations (in the genes OCA2, HERC2, SLC45A2, SLC24A4, IRF4, TYR, TYRP1, ASIP, and MC1R) for hair color, eye color, and freckling validate the Web-based, self-reporting paradigm. The identification of novel associations for hair morphology (rs17646946, near TCHH; rs7349332, near WNT10A; and rs1556547, near OFCC1), freckling (rs2153271, in BNC2), the ability to smell the methanethiol produced after eating asparagus (rs4481887, near OR2M7), and photic sneeze reflex (rs10427255, near ZEB2, and rs11856995, near NR2F2) illustrates the power of the approach.
... A Swedish blood donor study [5] investigated one of the largest representative samples of subjects and reported photic sneezing in about 24% of the examined subjects. Photic sneezing was also demonstrated as occurring in babies, suggesting that some kind of congenital factors might influence this phenomenon67. Collie et al. [3] observed that the prevalence is higher in subjects with a family history of photic sneezing, and they therefore suggest an autosomal dominant inheritance, which they called the ''ACHOO'' (Autosomal Cholinergic Helio-Ophtalmologic Outburst) syndrome. ...
Exposure to bright light such as sunlight elicits a sneeze or prickling sensation in about one of every four individuals. This study presents the first scientific examination of this phenomenon, called 'the photic sneeze reflex'.
In the present experiment, 'photic sneezers' and controls were exposed to a standard checkerboard stimulus (block 1) and bright flashing lights (block 2) while their EEG (electro-encephalogram) was recorded. Remarkably, we found a generally enhanced excitability of the visual cortex (mainly in the cuneus) to visual stimuli in 'photic sneezers' compared with control subjects. In addition, a stronger prickling sensation in the nose of photic sneezers was found to be associated with activation in the insula and stronger activation in the secondary somatosensory cortex.
We propose that the photic sneeze phenomenon might be the consequence of higher sensitivity to visual stimuli in the visual cortex and of co-activation of somatosensory areas. The 'photic sneeze reflex' is therefore not a classical reflex that occurs only at a brainstem or spinal cord level but, in stark contrast to many theories, involves also specific cortical areas.
Background: The photic sneeze reflex (PSR) is a widespread, yet understudied phenomenon characterised by sneezing in response to bright-light exposure, reportedly affecting around 30% of the general population. Our goals were to collect real-world data to characterise PSR-inducing naturalistic light conditions, and to develop an indoor protocol to reliably induce the PSR in affected subjects using parametric stimuli.
Methods: This study was carried out on one male adult affected by photic sneezing (n=1). To characterise naturalistic light conditions eliciting photic sneezing, real-world light exposure was measured over a 30-day period, while logging PSR events. To study photic sneezing in response to artificial stimuli, a setup including a multi-primary LED source and an integrating sphere was used to present 30-second light stimuli to the subject while collecting pupillometric data with an eye-tracker.
Results: 82 photic sneeze events were recorded, with an average of 2.73 sneezes per day and a range of 1 to 6 sneezes per event. At a sneeze event, illuminance is on average ten times bigger than five minutes before the sneeze event. A significant increase in illuminance is observed around 2 minutes before the sneeze event. Light levels go back down to pre-sneeze levels within 10 minutes after sneezing. Despite exposure to more than 150 stimuli, no sneeze could be artificially induced in the subject. However, a strong tickling sensation was consistently reported, especially for high illuminance settings.
Conclusions: Real-world light data confirmed that a sudden increase in environmental lighting conditions can induce photic sneezing. Further analysis could be relevant on instances of illuminance increments not eliciting a photic sneeze. The experimental setup only elicited tickling sensations, but with further testing and optimisation, it is reasonable to assume that it would reliably induce photic sneezes, thereby opening further mechanistic study of this intriguing phenomenon.
The prevalence of the photic sneeze response (PSR) as well as other characteristics have been studied in selected pop-ulations. However, the PSR has not been investigated in a general eye care patient population. This study was per-formed in an attempt to characterize the epidemiologic, descriptive, and demographic aspects of the photic sneeze response among patients attending for primary eye care. · · • · • · ··· • · • ·· • · · • oo • • • • • "·' "
Intractable sneezing is rare and may not be familiar to many physicians. A 10-year-old girl had a mild viral illness that culminated in uncontrollable sneezing without any other symptoms. She sneezed with her eyes either opened or closed, and her sleep usually was not interrupted by sneezing. Routine physical and rhinoscopic examination as well as laboratory investigations were normal. Multiple oral and topical medications were ineffective in providing long-term relief from sneezing. The patient had no obvious school or home problems, but evaluation by a child psychiatrist revealed significant internal conflicts. Educationally, she was a “gifted child,” yet she displayed infantile behavior, manifested by her continued sleeping with her parents. Family psychotherapy was recommended. Common features of psychogenic intractable sneezing are presence in an adolescent female, sneezing with eyes open, quiescence during sleep, nonresponse to medical therapy, normal physical examination, lack of related symptomatology, underlying psychologic stress, and good response to psychiatric techniques. Early consideration of a psychogenic origin and prompt psychiatric intervention for intractable sneezing may forestall exhaustive diagnostics and result in complete symptom resolution.
To investigate the frequency of sneezing among patients receiving intravenous sedation and periocular local anesthetic for oculoplastic procedures in a single center. To identify potential risk factors involved.
Prospective, consecutive, interventional case series in a single tertiary-referral oculoplastic unit.
A total of 294 patients undergoing 314 isolated oculoplastic procedures, performed under intravenous sedation with periocular local anesthetic from November 2007 to November 2008.
Prospective data collection on patient demographics, history of photic sneezing, intravenous sedative, depth of sedation, nasal oxygen, and periocular infiltration site. Standard local anesthetic was used in all cases, but the intravenous sedation was at the discretion of the attending anesthesiologist (7 in total).
Sneezing or attempted sneezing within 5 minutes of injection of the local anesthetic, as determined by agreed observation between attending staff.
Sneezing was observed in 16% of cases. No association was found between sneezing and patient age or presence of nasal oxygen. A weakly positive association was observed with male gender (55% sneezers vs. 37% non-sneezers, P = 0.03, relative risk [RR] = 1.5, confidence interval [CI], 1.1-2.0), bilateral infiltration (65% vs. 40%, P = 0.005, RR = 1.6, CI, 1.2-2.1), and upper eyelid infiltration (73% vs. 54%, P = 0.01, RR = 1.4, CI, 1.1-1.7). Photic sneezing was described in 47% of sneezers and 19% of non-sneezers (P = 0.0004, RR = 2.6, CI, 1.6-4.0). Because propofol was given to 95% of patients, no association with sneezing could be ascertained. However, opioid derivatives were found to be protective (12% vs. 43%, P<0.0001, RR = 0.3, CI, 0.1-0.6), whereas midazolam doubled the risk of sneezing (45% vs. 22%, P = 0.0008, RR = 2.1, CI, 1.4-3.0). Deep sedation (Ramsay score 5-6) also strongly increased the sneeze risk (65% vs. 23%, P<0.0001, RR = 2.8, CI, 2.1-3.8).
Propofol-based intravenous sedation, in combination with periocular local anesthetic injections, induces sneezing in approximately one sixth of general oculoplastic cases. Male gender, a history of photic sneezing, bilateral or upper eyelid infiltration, deep sedation, and the concurrent administration of midazolam all increased the risk, whereas adjunctive opioid use reduced the risk.
A sneeze reflex may occur after propofol sedation and during periocular injections. Unexpected movement from sneezing can result in needle injury to the globe or optic nerve, or hematoma. We investigate the efficacy of concomitant fentanyl or alfentanil in reducing sneezing following propofol and during periocular injections.
Our prospective, randomized study included 81 adult patients undergoing conscious sedation prior to periocular injection of local anesthesia. All patients received propofol and were randomized to propofol only (25 patients), propofol plus midazolam (14 patients), propofol plus opioid (31 patients), propofol plus midazolam and opioid (11 patients). Periocular injection of local anesthetic was given in the usual manner. The main outcome measure was sneezing. Patients were also assessed for cardiorespiratory parameters, analgesia, and adverse side effects of sedatives.
Of the patients who received no opioid, 17 of 39 (43.6%) sneezed. Of the patients who received an opioid, 0 of 42 sneezed (p < 0.0001 by Fisher exact two-tailed test). Among subjects receiving no opioids, midazolam was associated with a higher incidence of the sneeze reflex, but this was not quite statistically significant (p = 0.09). No adverse cardiorespiratory events were noted and analgesia was universally adequate.
Fentanyl or alfentanil suppressed sneezing after propofol sedation and eyelid anesthetic injection. These medications may prevent needle injury.
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Photic induced sneeze is a reflex that occurs in certain individuals after exposure to bright light. Cystinosis is an autosomal recessive inborn error of metabolism in which nonprotein cystine accumulates within lysosomes. The pathognomonic ocular manifestation of cystinosis is corneal crystal deposition. We observed photic induced sneezes during ophthalmoscopic examination in five of 19 patients with nephropathic cystinosis (26%). We report on this observation and discuss possible pathophysiological mechanisms for photic induced sneezing in cystinosis.
Sneezing in response to bright light has been found in 25% of a sample of the British population, and pedigrees are compatible with an autosomal dominant mode of inheritance. The mechanism of the response is discussed.
The prevalence of the photic sneeze response (PSR) as well as other characteristics have been studied in selected populations. However, the PSR has not been investigated in a general eye care patient population. This study was performed in an attempt to characterize the epidemiologic, descriptive, and demographic aspects of the photic sneeze response among patients attending for primary eye care.
A questionnaire on demographics, risk factors, and triggering stimuli was distributed to 500 consecutive patients presenting for a general eye examination at an academic health center optometry clinic.
Three hundred and sixty-seven of 500 questionnaires were returned (73.4%). Among this sample 33% were self-recognized photic sneezers with the majority being females (67%) and Caucasian (94.3%). Statistically significant correlations were found between the presence of photic sneezing and the presence of a deviated nasal septum and a non-significant association was found with tobacco use. Uniform frequency of sneezing does not occur in response to light stimulus; only 12.3% of sneezers responded consistently to sunlight exposure. The majority of sneezers (90.7%) responded with three or fewer sneezes. The interval between successive sneezes was fewer than 19 seconds in 85% of respondents. Fewer than 27% of respondents were able to recall a parent who exhibited a sneeze response.
The PSR is not an uncommon phenomenon. Systemic associations with the PSR are as diverse as deviated nasal septum and tobacco use. Results suggest that there may be a threshold level of light or frequency of light exposure which produces the response and that more patients may acquire the response than inherit it.
A literature review of reported cases of intractable sneezing reveals that intractable sneezing typically occurs in children or adolescents and is a manifestation of conversion disorder. Analysis of these cases demonstrates a number of features that are helpful in differentiating psychogenic from organic sneezing. Also discussed are aspects of patient management once a diagnosis of conversion disorder is established.
Prompted by the observation that a fellow medical student sneezed at about the same time in class every morning, we recorded the time of each of the 118 sneezes she made on 69 days over a 6.5-month period. Analysis of the sneeze times with the goodness-of-fit test revealed a highly nonuniform distribution (p < 0.0001) with a significantly increased probability of sneeze production within a small time interval centered at 8:20 AM. After ruling out the known causes of sneezing, we propose that the subject had a circadian fluctuation in her sneeze threshold independent of any immediate external stimulus. We compare this circadian rhythm to other periodic phenomena such as cyclic esotropia and rapid-cycling bipolar disease as well as to illnesses such as the periodic paralyses in which rapid and reversible changes occur in the neuromuscular system, and we discuss the peculiar circumstances favorable to documenting a circadian sneeze.
* BACKGROUND AND PURPOSE: Intravenous sedation to minimize discomfort from local anesthetic injection has many potential complications including severe involuntary sneezing (IS). This prospective study evaluates the occurrence of IS and a history of photic sneezing (PS).
* METHODS AND MATERIALS: All patients receiving local anesthesia (retrobulbar or periocular injections) after intravenous thiopentone for eye surgery during eight months were asked about PS and observed for IS.
* RESULTS: The 557 patients (40% males) had a mean age of 69.9 years and 14% recalled PS (29.5% males). IS developed in 5.2% of the 557- Only 7.6% of those with PS developed IS. After periocular injections 23-8% developed as compared to 4.5% after retrobulbar injections. (P<0.001). There was no relationship between PS and IS (p=0.43)
* CONCLUSION: IS is not linked to PS, with males and females at equal risk for either. IS is more common after periocular injections.
[Ophthalmic Surg and Lasers 1999;30:208-21 1.]
The photic sneeze response is a well-established clinical phenomenon in which patients respond by sneezing in an uncontrolled manner. This paroxysm of sneezing is evoked by a sudden exposure to an intense or bright light source. The pathogenesis of this response is apparently quite complex. Sudden exposure to bright light involves the optic, oculomotor, and trigeminal nerves, the autonomic nervous system and central brainstem functions. Anecdotal reports have suggested the photic sneezing response may occur as an autosomal dominantly inherited condition. However, such a familial pattern is not always present.
This article presents a descriptive report of a case in which a patient sought comprehensive eye care and, during the course of the examination, reported a relationship between light and a predictable auditory response. The mechanism for this involuntary auditory response to a bright light may be similar to that involved with photic sneeze response.
This patient's history, specific features of the auditory response, and ophthalmic findings as a result of the comprehensive eye examination are reported.
The photic sneeze response is not an uncommon phenomenon. A similar mechanism may explain the auditory response to a photic stimulus reported in this article.
Sneeze is an ubiquitous phenomenon that happens to everyone. In spite of this, little attention has been paid to it, among medical literature in general, and even less in neurologic texts. A curious entity, called autosomal dominant compelling helio-ophthalmic outburst syndrome, has been scarcely described in the scientific literature. This reflex appears when subjects are exposed suddenly to intense sunlight and it consists of long incoercible sneeze bursts. There are no publications on this subject among the spanish literature.and intensity increase with time.
To study the clinical and physiological features of the reflex in Spanish families.
Affected subjects were identified by personal interview and given a questionnaire drawn up for this study. Besides, they were instructed to give the questionnaire to their relatives.and intensity increase with time.
All the six families showed a high-penetrance, autosomal dominant inheritance. The reflex had a high consistency, a latency about 3 seconds, an intersneeze interval of 2 seconds and a frequency of 2-3 sneezes/ burst. Refractory period was long.and intensity increase with time.
Our study suggest a higher consistency, shorter latency and lower age of onset of the reflex in our patients than general population, and that frequency and intensity increase with time.
Sneeze is an ubiquitous phenomenon that happens to everyone. In spite of this, little attention has been paid to it, among medical literature in general, and even less in neurologic texts. A curious entity, called photic sneeze reflex, solar sneeze reflex, light sneeze reflex or autosomal dominant compelling helio-ophthalmic outburst syndrome, known perhaps since ancient Greek, has been scarcely described in the scientific literature, mainly as clinical notes and letters to the editor, but in a detailed way, we can find just a few reports. This reflex appears when subjects are exposed suddenly to intense sunlight and it consists of long incoercible sneeze bursts. It is usually ignored by its sufferers, who report it as a curiosity or a minor complaint, and its importance has been neglected in spite of its hereditary nature and its apparently high prevalence. We review the history, epidemiology, genetics, neuroanatomy, neurophysiology and physiopathology of this reflex hereditary response.
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