Timing and Duration of Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis for Robin Sequence.

OBJECTIVE: Collect data from craniofacial surgeons to analyze mandibular distraction osteogenesis (MDO) protocols, and facial nerve dysfunction (FND) to characterize this common, but poorly documented complication after MDO in infants with Robin Sequence (RS). DESIGN, SETTING, AND PARTICIPANTS: A 16-question anonymous survey designed through REDCap was digitally distributed to members of the American Cleft Palate-Craniofacial Association and International Society of Craniofacial Surgery (ISCFS). MAIN OUTCOME MEASURE(S): Demographic information, MDO perioperative variables, surgeon experience with FND after MDO for patients with RS, and the timing and duration of FND were analyzed. RESULTS: Eighty-four responses were collected, with 80 included for analysis. Almost two-thirds of respondent surgeons reported FND as a complication of MDO in patients with RS (51, 63.8%); 58.8% (n = 47) transient FND and 5% (n = 4) with permanent facial nerve palsy only. Both transient and permanent FND was documented by 13 (16.3%) respondents. Among respondents, FND was observed immediately following initial device placement/osteotomies in 45.1%, during distraction in 45.1%, during consolidation in 19.6%, and following device removal in 43.1%. Twenty-five of these respondent surgeons reported resolution of FND between 1 and 3 months (53.2%, n = 25). CONCLUSIONS: FND after MDO in patients with RS was noted by most respondents in this survey study. While most surgeons noted temporary FND, one-fifth reported long-term dysfunction. FND was documented most commonly following device placement/osteotomies or during active distraction. Further research should seek to establish risk factors associated with FND and identify surgical and perioperative prevention strategies.

Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis in Patients with Robin Sequence: A Systematic Review and Meta-Analysis.

OBJECTIVE: Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. DESIGN AND SETTING: According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: "distraction osteogenesis" and "Robin Sequence". A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. RESULTS: Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). CONCLUSION: This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.

Impact of Cochlear Dose on Hearing Preservation Following Stereotactic Radiosurgery in Treatment of Vestibular Schwannomas: A Multi-Center Study.

OBJECTIVE: Stereotactic radiosurgery (SRS) is a well-established treatment for vestibular schwannomas (VS). Hearing loss remains a main morbidity of VS and its treatments, including SRS. The effects of radiation parameters of SRS on hearing remain unknown. The goal of this study is to determine the effect of tumor volume, patient demographics, pretreatment hearing status, cochlear radiation dose, total tumor radiation dose, fractionation, and other radiotherapy parameters on hearing deterioration. METHODS: Multicenter retrospective analysis of 611 patients who underwent SRS for VS from 1990-2020 and had pre- and post-treatment audiograms. RESULTS: Pure tone averages (PTAs) increased and word recognition scores (WRSs) decreased in treated ears at 12-60 months while remaining stable in untreated ears. Higher baseline PTA, higher tumor radiation dose, higher maximum cochlear dose, and usage of single fraction resulted in higher post radiation PTA; WRS was only predicted by baseline WRS and age. Higher baseline PTA, single fraction treatment, higher tumor radiation dose, and higher maximum cochlear dose resulted in a faster deterioration in PTA. Below a maximum cochlear dose of 3 Gy, there were no statistically significant changes in PTA or WRS. CONCLUSIONS: Decline of hearing at one year in VS patients after SRS is directly related to maximum cochlear dose, single versus 3-fraction treatment, total tumor radiation dose, and baseline hearing level. The maximum safe cochlear dose for hearingtbrowd preservation at one year is 3 Gy, and the use of 3 fractions instead of one fraction was better at preserving hearing.

Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

OBJECTIVES: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. STUDY DESIGN: Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. RESULTS: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. CONCLUSION: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis.

OBJECTIVES AND MAIN OUTCOME MEASURES: To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity. STUDY DESIGN: Systematic Review and Meta-analysis. METHODS: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD. RESULTS: We identified 138 studies from the initial search, 12 of which met inclusion criteria and were utilized for data analysis. A total of 636 SCD patients and 360 controls were included in the Cochrane Review Manager 5.3 meta-analysis. There was a statistically significant increase in the prevalence of SNHL in adults with SCD compared with the general population with a cumulative risk ratio (RR) of 6.03. CONCLUSIONS: This is the first systematic investigation of the relationship between SCD and SNHL in adult patients across the globe. SNHL is more prevalent in patients with SCD, specifically those of the HbSS genotype, than the general population likely due to the pathophysiology of the disease and its effects on labyrinthine microvasculature. The increased prevalence of SNHL in the adult SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of adult SCD patients.

A Case of Incus Necrosis Following Oral and Intratympanic Steroid Administration for Sensorineural Hearing Loss.

OBJECTIVE: Intratympanic (IT) steroid injections are often used for the treatment of inner ear pathologies, including sudden sensorineural hearing loss. IT steroids are also commonly used in conjunction with or in case of systemic steroid therapy failure. They are viable options for patients who cannot endure side effects or are at high risk of complications from systemic therapy. The duration of treatment, strength, and type of steroid, and number of IT injections are important factors that impact possible treatment complications. Reported complications, though uncommon, include tympanic membrane perforation, otalgia, vertigo, otitis media, and acne. In this report, we explore a novel case of incus necrosis associated with oral and/or IT steroid administration. STUDY DESIGN: Clinical Capsule Report. SETTING: Tertiary Academic Center. PATIENT: We present a case of a 70-year-old male with sudden sensorineural hearing loss who developed incus necrosis and conductive hearing loss following treatment with oral and intratympanic steroids. INTERVENTION: The patient underwent a left tympanoplasty with ossicular chain reconstruction to treat the conductive component of his hearing loss. RESULTS: The left tympanoplasty almost fully-resolved the conductive component of his hearing loss. However, intraoperative and CT assessment of the temporal bone revealed resorption of the long process of the incus, with otherwise normal-appearing ossicles. CONCLUSION: We report the first case of possible steroid-induced necrosis of the long process of the incus. Owing to its tenuous blood supply, the incus may be predisposed to steroid-induced osteonecrosis similar to that of the femoral head.

Abnormal Cranial Shape Preceding Radiographic Evidence of Craniosynostosis.

Premature fusion of a cranial suture is known to result in characteristic changes in the head shape, even when the synostosis involves only part of the suture. We report an unusual case of a patient seen at the age of 2 months for an abnormal head shape that was present at birth. The phenotype was consistent with an isolated fusion of the frontosphenoidal suture, but the suture was open on a high-resolution computed tomography scan finding. There was no improvement in cranial form after 6 months of helmet therapy, and a follow-up computed tomography scan image taken at age 10 months showed the development of bilateral isolated frontosphenoidal synostosis. This case highlights that an abnormal head shape may, in some patients, predate radiographic evidence of craniosynostosis.