DOI: https://doi.org/10.36347/sjmcr.2026.v14i05.084

Pages: 1212-1217

Chudley-McCullough syndrome is a rare autosomal recessive disorder characterized by sensorineural hearing loss associated with distinctive brain malformations on magnetic resonance imaging. Published data on cochlear implantation in this condition remain limited. We report three sisters aged 18, 15, and 3 years with prelingual bilateral profound sensorineural hearing loss and a neuroradiologic pattern compatible with Chudley-McCullough syndrome, including corpus callosum abnormalities, parasagittal frontal polymicrogyria, heterotopia, and arachnoid cysts. Inner-ear structures and cochlear nerves were preserved in all three patients. All underwent unilateral cochlear implantation through mastoidectomy and posterior tympanotomy, with complete electrode insertion and no immediate postoperative complication. Follow-up was short, with postoperative assessments available at approximately 1 month in the eldest sister and approximately 3 months in the two younger sisters. Early auditory behavioral improvement was observed, particularly in the youngest patient, although outcome interpretation remains limited by the retrospective estimation of Categories of Auditory Performance and short follow-up. This familial series suggests that, in patients with profound hearing loss and imaging findings radiologically compatible with Chudley-McCullough syndrome, cochlear implantation may be considered when cochlear nerves are present and inner-ear anatomy is favorable. Molecular confirmation remains desirable for diagnostic consolidation and genetic counseling, but auditory rehabilitation should not necessarily be delayed solely for genetic testing when the clinical and radiological context is strongly suggestive.