Audiological profile in children with congenital inner ear anomalies

The current study aims to describe subjects and audiological profiles of children with congenital inner ear anomalies (IEAs).

A total of 193 children with sensorineural hearing loss (SNHL) and radiological evidence of one or more congenital IEAs were included.

The most common IEAs in the current study was enlarged vestibular aqueduct (EVA) either isolated or associated with other IEAs. Incomplete partition (IP) with its three types (IP I, IP II, and IP III) was the second common anomaly, followed by cochlear hypoplasia (CH). At the time of radiological diagnosis, hearing loss degrees ranged from mild to profound in children with EVA, CH III, and CH IV with the majority having severe or profound degrees. The prevalence of severe and profound degree of hearing loss was higher in children with IP than children with isolated EVA or CH III and CH IV. In children with isolated EVA, hearing loss was asymmetric in 52.2% and progressive in 58%. In children with CH III and CH IV, hearing loss was asymmetric but stationary. Only children with EVA and IP III had air-bone gab (ABG) at low frequencies, while children with other IEAs did not have ABG except if there was an association with EVA. Children with severe anomalies such as CH I, CH II, common cavity, and cochlear nerve hypoplasia had profound degrees of hearing loss or just sound detection.

Knowing the audiological profile of children with IEAs has important clinical implications in the management of those children.