Congenital Arrhinia with Profound Hearing Loss and Complete Cleft Palate: A Challenging Triad

Congenital arrhinia (CA) is an infrequent condition with hypoplasia of internal and external nasal passages. This, along with a complete cleft palate and profound hearing loss, constitutes a problematic challenge to rehabilitate the airway, speech and hearing. Aplasia of the nose is also associated with various other congenital anomalies, such as midline defects, olfactory agenesis and structural abnormalities in the brain. The patients are predisposed to respiratory and feeding problems at birth and need early intervention for the same. Bosma Arhinia Microphthalmia (BAM) syndrome is its most severe presentation. Rehabilitation is complicated and needs multidisciplinary cooperation. Due to its rare nature, management is decided on a case-by-case basis with no clearly defined guidelines. Sensory deprivation leads to delays in developmental milestones. To the authors' best knowledge, this is the first report of arrhinia with profound SNHL and cleft palate being reported in the literature.