Audiological profile in children with Incomplete Partition type I: case series

Abstract Introduction Incomplete Partition (IP) malformation constitutes 40% of Inner ear anomalies (IEAs) whereas the prevalence of IP-I was estimated to be around 20% of IEAs and around 2% of all cochlear malformation. IP-I has worse morphological and functional features than IP-II and IP-III. Early and accurate determination of type of IEAs is essential for early and appropriate intervention. Aim of the study To explore the audiological profile associated with IP-I anomaly and its importance to determine accurate and appropriate management. Patients and methods 14 children with radiological evidence of IP-I anomaly, who presented to the audiovestibular unit for 3 years (from 2023 to 2025) were included in the study. All children were subjected to full history taking, otoscopic examination, audiological evaluation including Immitancemetry, Pure tone audiometry, and Auditory brainstem response. Results Study group included 12 females and 2 males; their age ranged from 1to 18 years. Twelve of these children had bilateral IP-I with bilateral SNHL. Nearly half of the study group had IP-I associated with Enlarged vestibular aqueduct (EVA). Ten patients (71.43%) had bilateral profound hearing loss, two patients (14.29%) had bilateral severe hearing loss, and two patients (14.29%) had a unilateral hearing loss (moderately severe, profound hearing loss) and with very poor speech discrimination in majority of the children. Air bone gap (ABG) was present only in three children, those children had IP-I associated with EVA. Conclusion Children with IP-I anomaly present with a distinctive audiological profile characterized by predominantly bilateral profound SNHL and poor speech discrimination. The presence of lesser degree of hearing loss or pseudo-conductive components in cases associated with EVA further complicates the profile. Consequently, radiographic workup should be conducted when IEA is suspected.