How should I approach the management of a patient with achondroplasia who presents with recurrent ear infections?

In managing a patient with achondroplasia who presents with recurrent ear infections, a comprehensive and multidisciplinary approach is essential due to the increased risk of otitis media with effusion (OME) and related complications in this population. Initial management should include a thorough clinical assessment focusing on otoscopy, hearing testing, and tympanometry to confirm the presence of OME or other middle ear pathology, as children with achondroplasia are predisposed to craniofacial anomalies that increase the risk of middle ear dysfunction ¹ (Wright and Irving, 2012). Given the recurrent nature of infections, formal audiological assessment is important to evaluate any associated hearing loss, which can impact speech and language development and overall quality of life ¹.

Management should be tailored to the severity and impact of the ear infections and any hearing loss. Conservative strategies include monitoring and supportive measures such as minimizing exposure to tobacco smoke and environmental irritants, and advising on communication strategies to support hearing difficulties ¹. If OME-related hearing loss is confirmed and persistent, consider interventions such as air conduction hearing aids or bone conduction devices, especially if surgical options are contraindicated or delayed ¹.

Surgical management, including the insertion of grommets (ventilation tubes), should be considered for patients with persistent bilateral OME and hearing loss after a period of observation, typically around 3 months, or earlier if hearing difficulties significantly affect daily living ¹. When planning grommet insertion, adjuvant adenoidectomy may be considered unless there is an abnormality of the palate, which is relevant in achondroplasia due to potential craniofacial structural differences ¹. Risks and benefits of surgery, including potential complications such as tympanic membrane perforation and infection, should be discussed with the patient and family to enable shared decision-making ¹.

Multidisciplinary involvement is crucial, as achondroplasia patients often require coordinated care from ENT specialists, audiologists, speech and language therapists, and geneticists to address the complex interplay of skeletal and craniofacial abnormalities that contribute to recurrent ear infections and hearing loss (Kitoh et al., 2022). Regular follow-up and reassessment are important to monitor hearing status and the effectiveness of interventions, adapting management plans as needed ¹.