Hyperacusis in Williams syndrome (WS) is associated with a high-frequency hearing loss resembling the configuration of noise-induced hearing loss. The hyperacusis and hearing loss in WS may stem from a deficiency in the acoustic reflex resulting from auditory nerve dysfunction. Additional mechanisms that may mediate hyperacusis in WS and should be evaluated in future studies include recruitment, malformation of the facial canal, and haploinsufficiency of the elastin gene.

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Multiple ASSRs may reliably predict the behavioral threshold in subjects with SNHL and may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in candidates for cochlear implants and children with auditory neuropathy.

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Adenoidectomy alone is satisfactory treatment for nasal obstruction and obstructive sleep apnea in selected children. Though some adenoid regrowth is not rare, clinically significant adenoid regrowth is infrequent. Persistent or recurrent symptoms are attributable mainly to nasal pathology.

Congenital conductive hearing loss due to ossicular deformities can be treated by either rehabilitation with a hearing aid or surgical reconstruction. We present the results of exploratory tympanotomy performed in a large paediatric otolaryngology centre in 67 patients with non-serous congenital conductive hearing loss. Forty-two children had malformation of one or more ossicles without fixation of the stapes, and 19 had fixed stapes. In 26 cases, the surgeon decided not to perform surgical correction. Seven operated patients were lost to follow-up. As a group, 47 per cent of the patients who underwent reconstruction showed no significant benefit from surgery, with post-operative air-bone gaps (ABG) greater than 30 dB. Assessment of the results by pathology showed that 64 per cent of the patients with mobile stapes had an air-bone gap within 30 dB compared to only 33 per cent of the patients with fixed stapes. One patient sustained severe sensorineural loss after the procedure. Considering that exploratory tympanotomy is a relatively minimal, benign procedure but that findings during exploration may exclude the option of reconstruction (in 39 per cent of our patients), we suggest exploring the ear, but in a more realistic, informed way.