Otorhinologic Disorders in 22q11.2 Deletion Syndrome.

OBJECTIVE: Investigate incidence and natural history of otologic and sinonasal disease associated with 22q11.2 deletion syndrome. STUDY DESIGN: Case series. SETTING: Tertiary care children's hospital. METHODS: Charts from consecutive children born 2000 to 2018 with a diagnosis of 22q11.2 deletion, DiGeorge, or velocardiofacial syndrome based on the International Classification of Diseases (ICD)-9 and ICD-10 codes were reviewed. Otologic and rhinologic diagnoses and surgeries and immune and microbiologic laboratory findings were collected from the medical record. RESULTS: After the exclusion of patients with no 22q11.2 deletion (n = 101), otologic care at an outside hospital (n = 59), and loss to follow-up prior to 3 years of age (n = 22), 128 were included. Males comprised 80 (62.5%) patients, 115 (89.8%) were white, and the median age at genetic confirmation of 22q11.2 deletion was 119 days (range 0 days to 14.6 years). Recurrent acute otitis media (RAOM), chronic otitis media with effusion, chronic rhinosinusitis, and recurrent acute sinusitis were diagnosed in 54 (42.2%), 37 (28.9%), 10 (7.8%), and 8 (6.3%), respectively. Tympanostomy tubes were placed in 49 (38.3%). Adenoidectomy and sinus surgery were performed in 38 (29.7%) and 4 (3.1%), respectively. Neither immunoglobulin nor cluster of differentiation deficiency increased the odds of RAOM diagnosis, tympanostomy tube placement, or chronic/recurrent sinusitis. Methicillin-resistant Staphylococcus aureus was the most common organism in sinus cultures (4/13, 30.8%). Streptococcus pneumonia dominated otorrhea cultures (11/21, 52.4%). CONCLUSION: Approximately half of children with 22q11.2 deletion may experience otologic disease that often requires surgical management. Future studies will utilize a larger cohort to examine the role of immunodeficiency in otologic and rhinologic disease in this population.

The limits of pandemic precautions: Tympanostomy tube placement in children with cleft palate during COVID-19.

PURPOSE: Coronavirus Disease-2019 (COVID-19) mitigation measures have led to a sustained reduction in tympanostomy tube (TT) placement in the general population. The present aim was to determine if TT placement has also decreased in children at risk for chronic otitis media with effusion (COME), such as those with cleft palate (CP). MATERIALS AND METHODS: A cohort study with medical record review was performed including consecutive children, ages 0-17 years, undergoing primary palatoplasty at a tertiary children's hospital February 2019-January 2020 (pre-COVID) or May 2020-April 2021 (COVID). Revision palatoplasty (n = 29) was excluded. Patient characteristics and middle ear status pre-operatively and at palatoplasty were compared between groups using logistic regression or Wilcoxon rank-sum. RESULTS: The pre-COVID and COVID cohorts included 73 and 87 patients, respectively. Seventy (44%) were female and median age at palatoplasty was 13.5 months for CP ± cleft lip (CP ± L) and 5.5 years for submucous cleft palate (SMCP). In patients with CP ± L, TT were placed or in place and patent at palatoplasty in 28/38 (74%) pre-COVID and 37/50 (74%) during COVID (P = 0.97). In patients with SMCP, these proportions were 5/35 (14%) and 6/37 (16%), respectively (P = 0.82). Examining only patients <2 years of age also revealed no difference in TT placement pre-COVID versus COVID (P = 0.99). Finally, the prevalence and type of effusion during COVID was similar to pre-COVID. CONCLUSIONS: Reduced infectious exposure has not decreased TT placement or effusion at palatoplasty. Future work could focus on non-infectious immunologic factors underlying the maintenance of COME in these children.