Hearing loss and cochlear implantation in Chudley McCullough syndrome: A case series

To present a case series of patients with Chudley - McCullough syndrome (CMS) and provide audiometric outcomes pre - and post - cochlear implantation.

A retrospective case series was written based on six patients with CMS and hearing loss. Patients were treated in a tertiary-care medical center for their hearing loss with hearing aid fitting and cochlear implantation. Audiometric outcomes pre- and post-cochlear implantation were analyzed.

Three out of six patients were diagnosed with auditory neuropathy spectrum disorder (ANSD), one patient was suspected of ANSD, and in two patients, the presence of ANSD could neither be confirmed nor excluded. All patients were fitted with hearing aids, and all had limited benefit. In most cases, hearing deteriorated rapidly and eventually, all patients received a cochlear implant (CI), unilateral or bilateral. In general, aided thresholds with CIs were satisfactory. However, speech recognition varied widely between patients and was, on average, worse compared to patients with sensorineural hearing loss without CMS.

CMS was often diagnosed relatively late during childhood, and sometimes hearing loss was the first apparent symptom. Hearing loss was found to be progressive, often not detected shortly after birth and often complicated by ANSD. Cochlear implantation emerged as the optimal treatment, demonstrating superiority over hearing aid rehabilitation to improve hearing performance. Auditory and speech-language development outcomes remained poorer than in children with CI and without CMS. Based on these results, we advocate considering cochlear implantation early for children who have CMS and hearing loss.