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Hearing loss: Understanding auditory neuropathy in children

Auditory neuropathy (AN) is a term used to describe a type of hearing disorder in which the outer hair cell function (the cochlear amplifier) is…

Auditory neuropathy (AN) is a term used to describe a type of hearing disorder in which the outer hair cell function (the cochlear amplifier) is normal but with a disruption in conduction along the  hearing pathway. 

“The range of auditory complaints and auditory difficulties associated with Auditory Neuropathy is vast and has resulted in the acceptance of the term Auditory Neuropathy Spectrum Disorder (ANSD) to better describe the disorder,” said Dr. Samer SakkaAmini an Ear , Nose and Throat Specialist  at the Saudi German Hospital Dubai in a news release.

Dr Amini said majority of individuals with Auditory Neuropathy Spectrum Disorder have low frequency hearing loss and/or very poor speech recognition scores for the degree of hearing loss measured through Pure Tone Audiometry assessment. 

“ Poor temporal resolution is associated with Auditory Neuropathy Spectrum Disorder, which results in poor perception of changes in stimuli over time. Speech perception can be adversely affected due to reduced sensitivity to follow fast and slow temporal modulations, reduced ability to detect gaps in continuous sound, and reduced frequency discrimination at low frequencies. This in turn can lead to poor speech and language development skills,” Dr Amini said.

He said in  a particular case , when a three-year old boy (names withheld) was born, the routine newborn hearing screening suggested bilateral normal hearing. However, with increase in age, the child exhibited reduced response to name call and environmental sounds. Shortly after the birth, the child was diagnosed with hearing loss. Simultaneously, the development of speech and language skills was also hampered.

According to him , his concerned parents took him to some hospitals where he was diagnosed with  profound hearing loss and bilateral severe hearing loss. However, his mother believed that the medical reports might be wrong because of the child’s recent uncertain response to name call, environmental sounds and moderate-severe speech perception difficulties.

However, the mother’s observations where confirmed right at the Saudi German Hospital Dubai  where they went for second opinion, the statement said, as the boy was diagnosed and treated of Auditory neuropathy through advanced approaches and technologies deployed at the hospital.

“Our Audiology team began with non standardized method such as name calling and clapping at normal loudness levels. The child gave a constant and confirmed head turn response towards the source of sound. This made the team curious to give positive judgment over the mother’s observation, the statement said. 

Dr Amini said it was a rare condition coupled with several other symptoms like reduced speech and language output, fluctuating hearing and poor speech, and perception difficulty which the child exhibited. Thus, the child was diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD) and is now doing well after treatement..

The expert said Auditory Neuropathy Spectrum ANSD has only a prevalence of 0.23% to 15% in individuals with hearing loss. “Therefore, it is rare to encounter ANSD in the hospital setup, and may easily go unnoticed or unattended. Hence, it is important to carry out a detailed hearing assessment in a proper clinical setting.”

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