Emerging Clinical Features of Acute Mastoiditis in Israel: A Registry Based Cohort.

BACKGROUND AND OBJECTIVES: Acute mastoiditis (AM) is a severe infection in the young population, with possible life-threatening complications. This study aimed to characterize AM presentation, symptoms and signs, complications, and management, over a period of 10 years. METHODS: This large-scale population-based cohort studied "Clalit Health Care" records, to include patients <18 years diagnosed with AM, hospitalized between the years 2008-2018. After validation, we investigated clinical symptoms and signs, pneumococcal vaccination status, complications, laboratory and microbiological parameters, imaging, antibiotic treatment and surgical interventions. RESULTS: AM was diagnosed in 1189 patients, mean age of 2.71 years and 591 (49.71%) were female. Most presented with protrusion of pinna (83.1%), retro auricular redness (73.5%) and fever (71.8%). Patients <2 years of age had more symptoms (3.8 ± 1.4, opposed to 3.6 ± 1.5, P = 0.006) and showed higher white blood cell count and C-reactive protein values. Local and intracranial complications occurred in 233 (20.8%) and 75 (6.5%) patients, respectively. Complications were associated with increased white blood cell count and C-reactive protein and related to bacterial type, specifically Fusobacterium necrophorum (P < 0.0001), for which 50% had an intracranial complication. Between the years 2008-2018, Streptococcus pneumoniae-positive cultures decreased (30.9% to 10.3%, P > 0.0001) as opposed to group-A Streptococcus (10.9% to 30.9%, P = 0.002). CONCLUSIONS: This study shows a difference in AM appearance in the <2 years population and the association between white blood cell count, C-reactive protein and microbiology results with the occurrence of a complication. This may play a role in the management process, such as imaging and intervention needs. Although performed during the pneumococcal vaccine era, the disease microbiology was shown to change significantly throughout the study.

Risk factors for recurrent acute mastoiditis in pediatric patients: a registry-based cohort study.

OBJECTIVE: To describe characteristics of pediatric patients with recurrent acute mastoiditis, and to identify risk factors for this condition. STUDY DESIGN: A retrospective cohort study. SETTING: Data based on electronic medical records of the largest Health Maintenance Organization in Israel. METHODS: Children hospitalized due to acute mastoiditis during the years 2008-2018 were identified, and their diagnosis was verified. Patients with recurrent acute mastoiditis were identified and grouped, and their characteristics were outlined and compared to those of the original group to identify risk factors for recurrence. RESULTS: During the 11-year period, a total of 1115 cases of children hospitalized due to acute mastoiditis were identified with a weighted incidence rate of 7.8/100,000. Of this group, 57 patients were diagnosed with recurrence following a full clinical recovery. The incidence proportion of recurrent acute mastoiditis was 5.1% (57/1115), male-to-female ratio was 27:30, 73.4% were younger than 24 months, the median period from the first episode was 3.4 months (IQR 2.0;10.0), and 82.5% of the patients (n = 47) had a single recurrence, whereas 18.5% (n = 10) had two recurrences or more. Mastoidectomy and swelling over the mastoid area during the first episode were identified as the main risk factors for recurrent mastoiditis HR = 4.7 [(2.7-8.2), p < 0.001] and HR = 2.55 [(1.4-4.8), p = 0.003], respectively. Mastoidectomy was the only independent significant risk factor for recurrence in a multivariate analysis. CONCLUSIONS: Mastoidectomy and swelling over the mastoid area during the first episode of acute mastoiditis were found strongly related independent risk factor for future recurrent episodes of acute mastoiditis.

Rhinitis medicamentosa - comparing two treatment strategies: a retrospective analysis.

OBJECTIVE: Rhinitis medicamentosa poses a therapeutic challenge for both patients and physicians. Treatment strategies vary, starting with avoidance of decongestants, followed by medications or surgical intervention. This study aimed to compare two treatment strategies for this condition. METHODS: A review was conducted of patients diagnosed with rhinitis medicamentosa from 2013 to 2021, who were managed conservatively with medications or surgically by inferior turbinate reduction. RESULTS: Forty-seven patients were included: 21 patients were treated conservatively and 26 underwent turbinate reduction. Following surgical therapy, the frequency of using decongestants was significantly reduced (p < 0.001), with a significant improvement in Sino-Nasal Outcome Test-22 scores (p < 0.001). The conservative treatment group was significantly older with more co-morbidities. Following medical therapy, the conservative treatment group had a significant decrease in the frequency of decongestant use, but there was no significant improvement in their Sino-Nasal Outcome Test-22 scores. CONCLUSION: Compared to conservative treatment, inferior turbinate reduction for rhinitis medicamentosa resulted in reduced decongestant use and improved quality of life.

Xylitol nasal spray for prevention of recurrent acute otitis media in children: A prospective two-center cohort study.

OBJECTIVES: The purpose of this study was to assess the effectiveness and safety of xylitol nasal spray as a prophylactic treatment for children with recurrent acute otitis media (AOM). METHODS: This is a prospective pilot study of children aged 1-4 years, diagnosed with recurrent AOM (at least three episodes in the three months before recruitment) between December 1, 2019 and January 31, 2023. Children were treated with nasal xylitol spray 2-3 times daily for 3 months. The number of AOM episodes and treatments administered were compared within 3-month intervals: before recruitment, during xylitol use, and during the three subsequent months. RESULTS: Of 68 children enrolled, 66 (97%) completed the follow-up, until July 2023. Thirty-eight (58%) were males. Sixty-three children (95%) were 12-24-months old. The mean number of AOM episodes during xylitol use, 1.06 (95% confidence interval [CI]: 0.73-1.39), was lower than in the 3-month previous interval, 4.12 (95% CI: 3.89-4.40), p < 0.001; and similar to that in the subsequent 3-month interval, 0.79 (95% CI: 0.49-1.08), p = 0.082. A similar pattern was observed in an analysis of the number of AOM episodes per patient month. The data were similar during spring and summer months as during autumn and winter months. Across the consecutive three-month intervals, decreases were observed in the mean number of AOM episodes treated with systemic antibiotics (3.35, 0.65, and 0.41), p < 0.001; and with topical antibiotics (1.38, 0.55, and 0.32), p < 0.001. No major side effects were recorded. CONCLUSIONS: The findings support the effectiveness and safety of nasal xylitol spray, for preventing recurrent AOM in children aged 1-4 years.

Epidemiology of pediatric acute mastoiditis in Israel: A National Registry 10-year perspective.

Background and Objectives: Previous small studies have proposed a higher incidence of acute mastoiditis in Israeli pediatric patients than in other Western countries. The aim of this study was to describe the incidence of acute mastoiditis and its epidemiological features over a decade, in order to identify variables that could possibly affect the incidence. Methods: All admitted patients aged <18 years diagnosed with acute mastoiditis between 2008 and 2018 at Clalit Healthcare Services were identified and a database was generated. Results: A total of 1189 and 1115 patients met the inclusion criteria, respectively. Acute mastoiditis diagnosis was confirmed in 95.2% of the patients. The incidence was 7.78 cases per 100,000 children-years but was significantly higher in children under 2 years of age (average of 38.31 per 100,000 children-years). No specific pattern was observed in the annualized incidence rate during the study period. Acute mastoiditis was significantly more common in children of Jewish descent than non-Jewish (10.4 vs. 3.03 per 100,000 children-years, P < 0.001) and of high socioeconomic status and is more common in the winter. The prevalence of household parental smoking (52%) was more than double that previously reported in the Israeli population. Conclusions: A higher incidence of acute mastoiditis was observed in the Israeli population than in other reports. The age-dependent rate was identified along with unique epidemiological features such as seasonality, higher incidence in patients of Jewish descent, or high socioeconomic status. Related parental smoking habits lend further support against the exposure of young children to household smoking.Level of evidence: Individual retrospective cohort study.

Laboratory and imaging findings of necrotizing otitis externa are associated with pathogen type and disease outcome: A retrospective analysis.

OBJECTIVE: To examine associations of laboratory and imaging data with diagnostic parameters of necrotizing otitis externa (NOE) and its severity, and to compare between bacterial and fungal infections. METHODS: Records of patients diagnosed with NOE during 2010-2018 at the Department of Otolaryngology, Head and Neck Surgery were reviewed retrospectively for demographics; disease characteristics; and laboratory, scintigraphy, and imaging results. RESULTS: Of 48 patients with NOE, the mean age is 73±11.6 years; 32 (67%) were males; 83% had diabetes mellitus. Common pathogens were pseudomonas (49%) and fungi (33%). Sensitivities of the technetium-scan (SPECT ratio ≥1.5), temporal bone computed tomography (CT), and gallium-scan (SPECT ratio ≥1.3) were: 78.7%, 48.8%, and 31.4%, respectively. Gallium-scan results correlated positively with CT bone involvement (p=0.002) and hospital length of stay (p=0.0014). C-reactive protein (CRP) level correlated with hospital length of stay (p=0.028) and positive technetium-scan results (p=0.012). Fungal infection had a higher technetium SPECT ratio (2.16 vs. 1.77, p=0.04), gallium SPECT ratio (1.4 vs. 1.2, p=0.02), longer duration of systemic treatment (87.4 vs. 37.9 days, p=0.014), and longer hospital length of stay (31.6 vs. 15.2 days, p=0.004) compared to non-fungal infection. Eight (17%) patients had responded poorly to treatment. Fungal pathogens, facial nerve paresis, extra-auricular, and bilateral disease were more prevalent among the non-responders. CONCLUSION: The technetium scan has higher sensitivity than temporal bone CT for diagnosing NOE. The gallium scan and CRP correlated well with hospital length of stay. A high rate of fungal infection was found, with significantly higher technetium and gallium SPECT ratios and worse outcome compared to bacterial infection. Fungal NOE remains therapeutically challenging.

Is Nasal Endoscopy of Diagnostic Value in Chronic Rhinosinusitis Without Nasal Polyps?

Endoscopic examination of the nasal cavity is an integral part of the assessment of patients with chronic rhinosinusitis. However, the benefit gained by performing endoscopy with respect to the patient's medical condition has not been completely assessed. We conducted a prospective cohort in an academic medical center. Thirty-nine patients diagnosed with chronic rhinosinusitis, without polyps, whose ailment was managed conservatively with no surgical intervention. All patients underwent nasal endoscopy, a computed tomography (CT) scan rated for Lund-MacKay score, and completed a sino nasal outcome test (SNOT)-20 questionnaire. This same evaluation was repeated 6 weeks following medical treatment. The CT scan and SNOT-20 questionnaire were independent parameters to evaluate the endoscopy score in each nasal passage. Thirty-nine (28 females) patients completed the follow-up period. A total of 156 endoscopic evaluations were performed, in which 74 nasal cavities were found to have significant pathology and 82 were either normal or displayed mild pathology. The correlation with the Lund-MacKay score was poor with a positive predictive value of 68.9% (31.1% false positive and 28% false negative). However, while looking at the SNOT-20 score, corrected for repeated measures, a higher endoscopy score was associated with a higher SNOT-20 score (odds ratio = 3.53, 95% confidence interval = 1.54-8.09, P = .003). Patients with higher endoscopy scores had a greater probability for exhibiting severe symptoms. However, we could not demonstrate a strong correlation between nasal endoscopy findings and CT scan scores. Therefore, with respect to patients with chronic rhinosinusitis without polyps, nasal endoscopy could be utilized as a beneficial objective tool.

[OTOLARYNGOLOGY, HEAD AND NECK SURGERY - VARIETY OF PATHOLOGIES AND MANAGEMENT OPTIONS].

INTRODUCTION: The field of otolaryngology, head and neck surgery faces a wide range of medical conditions, starting with upper airway morbidity, head and neck tumors, hearing loss and deafness, ear diseases, nose, sinuses, and anterior skull base conditions, pediatric otolaryngology, laryngology and facial and reconstructive plastic surgery. The current issue of this journal presents a variety of manuscripts that are in the forefront of our specialty in Israel and around the world. The management of laryngeal tumors is undergoing thorough modification aiming to preserve the organ while early tumors are treated endoscopically using laser technology. The human papilloma virus is one of the major changes that recently emerged as an important etiologic factor for oropharyngeal carcinoma in young nonsmokers. Hearing loss and deafness are negative contributors to the quality of life. Congenital hearing loss has a major effect on brain development, and speech and language development, therefore, early cochlear implantation is beneficial for well child development. Different aspects associated with cochlear implantation are discussed in this issue. Many fields that are associated with our specialty are gradually utilized by otolaryngologists, for example the utilization of ultrasound by surgeons. The field of otolaryngology head and neck surgery is dynamic and abundant with innovative technologies that are presented in this article, starting with cochlear implants, robotic surgery, and the utilization of 3D printers for complicated facial reconstructions. We are in the midst of a journey and we may assume that the future holds great promise both for our patients and for us as health care providers.

Adenoid Obstruction Assessment in Children: Clinical Evaluation Versus Endoscopy and Radiography.

BACKGROUND: Hypertrophy of the adenoids is common in children. However, the anatomical site makes the adenoids difficult to assess, and studies evaluating the subject are ambiguous, especially with regard to the use of X-ray as an evaluation tool. OBJECTIVES: To compare medical history with clinical, radiological, and endoscopic evaluations of the adenoids and compare obstructed and non-obstructed children relative to the assessment methods. METHODS: A prospective comparative study was conducted with children who were suspected of having enlarged adenoids. All parents completed a medical history questionnaire and patients underwent clinical evaluation based on Nasal Obstruction Index (NOI) scores, radiological assessment based on the lateral neck X-ray adenoid-nasopharynx (A/N( ratio, and endoscopic evaluation based on anatomical relations. Spearman correlations were used for comparison between methods. RESULTS: We evaluated 36 patients, median age 5.33 years. Correlation measurements for clinical assessment and questionnaire (r = 0.582, P < 0.0001), questionnaire and endoscopy (r = 0.462, P = 0.005), and clinical assessment and nasal endoscopy (r = 0.621, P < 0.0001) were statistically significant. None of the parameters correlated with the radiological findings. A statistically significant difference was found between the obstructed and non-obstructed groups in both questionnaire (P = 0.004) and clinical assessment (P < 0.0001). However, no difference was found in X-ray measurements. CONCLUSIONS: Lateral neck X-ray measurements were not correlated to symptoms, signs, or endoscopic findings. Therefore, medical professionals should use lateral neck radiography when considering adenoidectomy only on a highly selective basis.

The role of surgery in necrotizing otitis externa.

This retrospective case review describes a subset of 5 patients with necrotizing otitis externa (NOE) with a refractory disease course who underwent surgery as part of their management plan between 2008 and 2013. Surgery promoted the cure of 4 of the 5 patients, and a fungal pathogen was recovered in 4 of 5 surgical samples. We conclude that surgery may be a necessary diagnostic and treatment adjunct in selective cases of NOE, especially in patients with a refractory disease course or with a suspected fungal etiology.

[CLINICAL APPROACH TO PEDIATRIC SEROUS OTITIS MEDIA].

Serous otitis media (also known as otitis media with effusion) is one of the most prevalent pediatric diagnoses. However, the recommended clinical approach and significance of this entity are controversial. Pathogenesis is usually based upon a combination of factors as overviewed in the body of the article. The cognitive and behavioral effects amongst children suffering serous otitis media were extensively studied and data points to little if any effects during long term follow-ups in otherwise healthy children. The therapeutic approach can be divided into watchful waiting, systemic drugs, topical drugs, mechanical therapies and surgical therapy (i.e. ventilation tube insertion). The reviewed literature mainly supports the effectiveness of the surgical approach in carefully selected cohorts of patients.

Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation.

Clinical utility of a polymerase chain reaction assay in culture-negative necrotizing otitis externa.

OBJECTIVE: This study describes a subset of necrotizing otitis externa (NOE) patients with a refractory disease and negative cultures. In these cases, we decided to use a polymerase chain reaction (PCR) assay from surgically obtained tissue under sterile conditions to improve pathogen detection sensitivity. STUDY DESIGN: Retrospective case review. SETTING: Academic medical center. PATIENTS: Nineteen consecutive patients diagnosed with NOE between January 2008 and January 2014 inclusive. Three patients of this cohort presented a culture-negative disease. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: Positive detection of pathogens using a PCR assay in cases with a complicated course of NOE and clinical resolution of the disease after targeted therapy according to PCR results. RESULTS: Surgical samples were obtained under sterile conditions from three patients with negative cultures and a refractory disease course of NOE. PCR assays were performed using pan-bacteria and pan-fungi protocols. In all three samples, a positive result for a fungal pathogen was recorded and followed by successful empirical targeted therapy. CONCLUSION: Patients who present with a refractory culture-negative NOE should be suspected as suffering from a fungal disease. The PCR assay may be an important laboratory adjunct in detecting pathogens responsible for NOE and can aid to promote therapy and disease resolution.

Use of complementary alternative medicine in pediatric otolaryngology patients: a survey.

OBJECTIVE: Limited data are available about complementary alternative medicine (CAM) use in children attending otolaryngology services. We investigated the pattern of CAM use among children and adolescents attending a pediatric otolaryngology clinic. METHODS: A cross-sectional survey. Anonymous questionnaires were administered, prior to doctor's admission, to parents accompanying young patients attending the pediatric otolaryngology clinic. Parents were asked about their general attitude toward CAM and whether they had ever consulted or considered a consultation with a CAM therapist. Subsequently, CAM users were asked to provide details on CAM modalities used and on their overall satisfaction with CAM therapy. RESULTS: Of 308 questionnaires administered, 294 parents responded (95% response rate). Ninety-four parents (32%) reported considering CAM, or previous or current CAM use. Commonly used CAM treatments were acupuncture (44%), homeopathy (36%), and naturopathy (6.7%). CAM users assessed success rate as being: successful (37%), unsuccessful (24%) or undetermined (39%). Successful treatment was described in terms of cure, improvement and better awareness of the problem being addressed. In most cases parents stated that the primary physician was aware of CAM use by the child (74%). CONCLUSIONS: CAM plays a substantial role among parents of children referred to pediatric otolaryngology consultation. The otolaryngologist awareness of parents' preference and interest may contribute to decision making regarding pediatric patients' management. Further investigations regarding CAM are warranted and clinical collaboration with CAM therapists should be considered.

Vertigo in children and adolescents: characteristics and outcome.

OBJECTIVES: To describe the characteristics and outcome of vertigo in a pediatric population. PATIENTS: All children and adolescents presenting with vertigo to a tertiary otoneurology clinic between the years 2003-2010 were included in the study. RESULTS: Thirty-seven patients with a mean age of 14 years were evaluated. The most common etiology was migraine-associated vertigo (MAV) followed by acute labyrinthitis/neuritis and psychogenic dizziness. Ten patients (27%) had pathological findings on the otoneurological examination. Abnormal findings were documented in sixteen of the twenty-three (70%) completed electronystagmography evaluations. Twenty patients (54%) were referred to treatment by other disciplines than otology/otoneurology. A follow-up questionnaire was filled by twenty six (70%) of the study participants. While all patients diagnosed with MAV had continuous symptoms, most other patients had complete resolution. CONCLUSIONS: Various etiologies of vertigo may present with similar symptoms and signs in the pediatric patient. Yet, variable clinical courses should be anticipated, depending on the specific etiology. This is the reason why treatment and follow up should be specifically tailored for each case according to the diagnosis. Close collaboration with other medical disciplines is often required to reach the correct diagnosis and treatment while avoiding unnecessary laboratory examinations.

[Chronic rhinitis--clinical guidelines 2010].

Chronic rhinitis (CR) affects large populations worldwide, diagnosed in 40% of the population and is often associated with co-morbidities, such as asthma, sinusitis, rhinoconjunctivitis cognitive dysfunction, and leads to loss of work and school days. Recently, global clinical guidelines were proposed for allergic rhinitis (AR). Similarly, an Israeli expert panel, consisting of ENT and Allergy/Immunology specialists, was delegated to discuss the current approaches to CR (including allergic and non-allergic rhinitis) and to submit a consensus paper. The guidelines, briefly presented herein, describe the epidemiology, mechanisms, diagnostic procedures, treatment modalities and clinical-management algorithms. It is adapted to the Israeli health system and will be distributed to all physicians by the Israel Medical Association. The new clinical guidelines are expected to update current knowledge, improve communication between medical disciplines, provide medico-legal support and improve the management of CR.

Angiomyomatous hamartoma in a submandibular lymph node: a case report.

Angiomyomatous hamartomas have been found almost exclusively in the inguinal and femoral lymph nodes; few reports of these lesions in the head and neck region have been published. We present a case of angiomyomatous hamartoma in the submandibular area, a site that has not been previously reported in the literature. The mass was initially diagnosed as an enlarged lymph node. When it did not regress following broad-spectrum antibiotic treatment, the patient, a 51-year-old woman, underwent an excisional biopsy. Histopathology identified the mass as an angiomyomatous hamartoma. At follow-up 3 years and 9 months postoperatively, the patient exhibited no evidence of recurrence on physical examination and computed tomography. Even though angiomyomatous hamartoma of the head and neck is rare, we suggest that otolaryngologists include it in the differential diagnosis of head and neck masses.

Epinephrine/Lidocaine injection vs. saline during endoscopic sinus surgery.

OBJECTIVES: To assess the safety and effectiveness of an epinephrine/lidocaine mixture administered by injection versus epinephrine administered topically and to learn its pharmacokinetics following administration to the nasal mucosa. DESIGN: A double-blind randomized controlled trial. METHODS: Patients were assigned into two groups and were injected with either epinephrine 1:100,000 and lidocaine 1% or saline alone during endoscopic nasal surgery under general anesthesia. Pledgets soaked in epinephrine 1:1,000 were used throughout the procedure in both groups. Hemodynamic measurements and catecholamine blood levels were obtained. RESULTS: Ten patients were randomized to the epinephrine group and 12 to the saline group. We were able to measure epinephrine and norepinephrine levels following injection in all patients. Epinephrine levels were similar in both groups immediately after injection; however, 15 minutes following injection, epinephrine was significantly higher in saline-injected patients. Mean arterial pressure and heart rate were affected by epinephrine and norepinephrine levels immediately after injection but were never elevated over the normal range. Heart rate was higher (P < .05) in the saline injected group than in the epinephrine group throughout the measurement period. The surgeons believed that the surgical field was bloodier in saline-injected patients (P < .05) however objective estimation of blood loss showed no difference. CONCLUSIONS: Injection of epinephrine/lidocaine mixture does not produce higher blood levels of epinephrine when compared to saline injection and did not induce any harmful side effects. We postulate that the combination with lidocaine 1% may reduce the patients' stress and thus prevent higher catecholamine levels.

Aneurysmal bone cysts of the head and neck in pediatric patients: a case series.

OBJECTIVES: To characterize aneurysmal bone cysts (ABCs) of the head and neck as seen at a tertiary care center. DESIGN: A case series. METHODS: A retrospective chart review of pediatric patients with aneurysmal bone cyst of the head and neck treated at the Hospital for Sick Children during the years 1994-2006 was carried out. RESULTS: Nine patients with ABC were treated. The average age at diagnosis was 9.3 years (range: 5 months-15 years), six males and three females. Four cysts originated in the paranasal sinuses, two in the mandible, one in the zygoma, one in the mastoid and one in the parietal bone. Two cases were associated with a previous localized trauma (mandible, zygoma). 5/9 presented with pain, 4/9 presented as an asymptomatic mass, 2/9 were associated with proptosis and nasal obstruction, 2/9 with nasal obstruction and 1/9 presented with an aural polyp. Seven cysts were successfully excised while one (in the pterygomaxillary fossa) is being followed conservatively. In one case the ABC was found to be secondary to an extensive osteoblastoma and this child continuous to be problematic. Follow-up time ranged from 2 years and 6 months to 10 years. CONCLUSIONS: ABC of the head and neck may vary in presentation and severity. Surgical removal is the treatment of choice at our institution and may provide a satisfying outcome. ABC may be secondary to an underlying bone pathology (e.g., osteoblastoma) which may make it refractory to treatment.

Hearing in children with fetal alcohol spectrum disorder (FASD).

BACKGROUND: Alcohol is the most prevalent human teratogen affected by early exposure of the fetus. Although not listed as a major part of the fetal alcohol spectrum disorder (FASD), different texts list hearing loss as a characteristic of the FASD, based on several small studies. OBJECTIVE: To characterize hearing in children with FASD, diagnosed in the Motherisk Program in Toronto. DESIGN: Cross sectional cohort study. SETTING: Academic referral center. PATIENTS: Children 4-16 years of age that met criteria for FASD, with no other known risk factor for sensorineural hearing loss. A consecutive sample of 41 children (13 girls, mean age 8.9 +/-3 years) was collected. INTERVENTION: Physical examination, audiometry and tympanometry. OUTCOME MEASURES: External and middle ear pathology on physical examination, pure tone average (PTA), speech reception threshold (SRT), discrimination and tympanometry. Results were compared to reference values in the normal population. Hearing loss equal or greater than 16dB hearing-level in each frequency tested was considered to be clinically significant. RESULTS: A total of 5 (11.2%) of children had hearing loss of at least 16dB hearing-level, mostly unilateral. SRT was within the normal range in 40 (98%) of children with FASD and discrimination was normal in all children. None had auricular or external canal dysmorphology. 14.7% of the children had frequent episodes of acute otitis media. Middle ear effusion was detected in 8 ears (9.8%). CONCLUSIONS: The prevalence of mild sensorineural hearing loss in children diagnosed with FASD (16dB hearing-level or greater) was not higher than expected in this age group. However, because children with FASD are academically and behaviorally challenged, early detection of hearing loss and early intervention is warranted.