Does microtia predict severity of temporal bone CT abnormalities in children with persistent conductive hearing loss?

Division of Pediatric Radiology, Department of Radiology and Radiological Science, Johns Hopkins Hospital, 600 North Wolfe Street, Nelson Basement, B-172, Baltimore, MD 21287-0842, United States. Electronic address: .
Journal of Neuroradiology (Impact Factor: 1.75). 02/2013; 40(3). DOI: 10.1016/j.neurad.2012.10.002
Source: PubMed


This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia.

Patients and methods:
From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience.

Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL.

Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes.

17 Reads
  • [Show abstract] [Hide abstract]
    ABSTRACT: A subset of patients presents with unilateral conductive hearing loss, a normal pinna or grade I microtia, and mild external auditory canal stenosis. The physical findings of microtia and a small external canal are commonly absent or subtle in this group of patients, who are being commonly referred for imaging to evaluate isolated conductive hearing loss. We present a case series of patients with unilateral conductive hearing loss and characteristic ossicular abnormalities, commonly anterior fixation of the malleus. All patients had a significantly increased distance from the cochlear promontory to the handle of the malleus and an abnormal incudostapedial angle, indicative of an abnormal ossicular position and/or morphology. Successful surgical reconstruction of the ossicular chain was attempted and accomplished in 3 patients.
    No preview · Article · Aug 2014 · American Journal of Neuroradiology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Microtia is hypoplasia of pinna that ranges in severity from partial to complete aplasia. Congenital aural atresia (CAA) is one of the most prevalent anomalies recognized with microtia. A 2-month-old male mix dog was referred to the veterinary animal hospital of Shahid Bahonar University of Kerman for vaccination. Clinical examination revealed hypoplasia of pinna in the right ear, whereas the pinna was normal on the left side. On otoscopic examination and radiology, no abnormality was found in the auditory canal and tympanic membrane of affected ear except narrowing compared with the other side. Unilateral congenital microtia and aural atresia were diagnosed. This report is the first description of microtia in dog.
    No preview · Article · May 2015 · Comparative Clinical Pathology