A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing...

Area of Science:

• Otolaryngology
• Pediatric Audiology
• Neuroscience

Background:

• Congenital deafness due to inner ear malformations often results in profound hearing loss and balance issues.
• Hearing aids are typically ineffective for such conditions, often requiring cochlear implants.
• Inner ear malformations include incomplete partition type I (IP-I) and common cavity deformity.

Purpose of the Study:

• To report a rare case of successful hearing aid use in a child with severe bilateral inner ear malformations.
• To investigate the auditory and vestibular function in a child with complex inner ear anomalies.
• To explore the potential for partial hair cell preservation in malformed inner ears.

Main Methods:

• Case report of a 6-year-old boy with congenital deafness and bilateral inner ear malformations (IP-I right, common cavity left).
• Assessment of hearing aid efficacy for hearing recovery.
• Electronystagmography and damped rotational chair testing for vestibular function evaluation.

Main Results:

• The patient experienced significant hearing recovery with hearing aids, enabling conversational ability.
• Electronystagmography revealed per-rotatory nystagmus in both directions.
• The patient demonstrated good equilibrium function despite severe malformations.

Conclusions:

• This case is rare, showing favorable hearing and balance development in a deaf child with severe bilateral inner ear malformations.
• Findings suggest possible partial preservation of auditory-vestibular hair cells.
• This challenges the typical expectation of poor outcomes in similar cases and warrants further investigation.