Deep neck abscess after intubation trauma: a case report.

Significant injuries from endotracheal intubation are exceedingly rare but can lead to life-threatening complications, such as pharyngeal perforation. This type of perforation can result in abscess formation and airway compromise. Risks for this complication include operator skill and intubation in emergent situations. This case report details a 59-year-old male who underwent elective septoplasty with bilateral nasal turbinate reduction. The procedure required general anesthesia induction and endotracheal intubation. He developed a gradually enlarging right-sided neck mass with associated fevers, neck pain, odynophagia, and dysphonia. He presented to the emergency department on postoperative day 5 and was diagnosed with a right-sided, prevertebral space abscess with airway mass effect secondary to pharyngeal perforation. He was admitted for operative management, intravenous antibiotics, and was successfully treated. While significant injury from endotracheal intubation is rare, it can result in infection and threaten airway patency. Emergency physicians must recognize pharyngeal perforation as a potential source of infection following instrumentation of the pharynx. This case has been reported to increase awareness of the potential for such injury.

Practicality and cost-effectiveness of using MRI compatible EEG system in the critical care setting.

BACKGROUND: Continuous video-EEG (cvEEG) monitoring is a vastly utilized tool for monitoring critically ill patients in the intensive care unit. Our study investigates the clinical utility and cost-effectiveness of using MRI Compatible EEG electrode system for patients being monitored in the intensive care unit. METHODS: This retrospective study included 14 critically ill patients who underwent cvEEG between March 2019 to March 2020. They were classified into 2 subgroups: Group 1- 'MRI-compatible EEG' (mean age: 56.00 ± 19.99 years; M:F = 2:5; N = 7), Group 2 - 'Conventional EEG' (mean age: 49.14 ± 24.76 years; M:F = 4:3; N = 7). The EEG monitoring times as well as cost-effectiveness of cvEEG between the groups were compared using Mann-Whitney Test (p ≤ 0.05). We also compared the MRI quality between the groups using Chi-squared test (p ≤ 0.05). RESULTS: The EEG non-monitored time in Group 2 (7.62 ± 6.45 h) was significantly higher than Group 1 (2.71 ± 2.34 h)] (p = 0.025). The average daily cost for cvEEG in Group 1 ($2098.53 ± 493.58) and Group 2 ($2230.58 ± 142.73) was comparable (p = 0.896). The quality of MRI scans between Group 1 (6/7) and 2 (6/7) were also comparable (p = 1.000). CONCLUSIONS: The monitoring time lost in patients with MRI Compatible EEG electrodes was significantly lower than the patients with Conventional EEG electrodes. The daily cost of monitoring and the quality of MRI scans were comparable between the 2 groups. We conclude that the use of MRI Compatible EEG electrodes is a practical and cost-effective method to improve the quality of monitoring in critically ill patients.

Sleep profile and Polysomnography in patients with drug-resistant temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) and the effect of epilepsy surgery on sleep-a prospective cohort study.

OBJECTIVES: We analyzed changes in sleep profile and architecture of patients with drug-resistant TLE-HS using three validated sleep questionnaires- Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), NIMHANS Comprehensive Sleep Disorders, and polysomnography (PSG). We studied the effect of epilepsy surgery in a subset of patients. METHODS: In this prospective observational cohort study, sleep profile of 40 patients with drug-resistant TLE-HS was compared to 40 healthy matched controls. Sleep architecture of 22 patients was studied by overnight PSG and compared to 22 matched controls. Sleep profile was reassessed in 20 patients after a minimum period of three months after epilepsy surgery. RESULTS: The mean PSQI was higher among patients compared to controls(P=0.0004) while mean ESS showed no difference. NCSDQ showed fewer patients feeling refreshed after a night's sleep compared to controls (p=0.006). PSG revealed a higher time in bed (p=0.0001), longer total sleep time (p=0.006) and more time spent in NREM stage 1 (p=0.001) and stage 2 (p=0.005) while spending less time in stage 3 (p=0.039) among TLE patients. Sleep efficiency was worse in patients on ≥3 ASMs compared to those on 2 ASMs (p-0.044). There was no change in mean ESS (p=0.48) or PSQI (p=0.105) after surgery. CONCLUSIONS: Patients with drug-resistant TLE-HS have an altered sleep profile and architecture. Patients on ≥3 ASMs have a lower sleep efficiency. Reassessment at short intervals after epilepsy surgery did not reveal significant changes in sleep profile.

NREM Sleep and Antiepileptic Medications Modulate Epileptiform Activity by Altering Cortical Synchrony.

INTRODUCTION: The activating role of non-rapid eye movement (NREM) sleep on epileptic cortex and conversely, the seizure remission brought about by antiepileptic medications, has been attributed to their effects on neuronal synchrony. This study aims to understand the role of neural synchrony of NREM sleep in promoting interictal epileptiform discharges (IEDs) in patients with epilepsy (PWE) by assessing the peri-IED phase synchrony during awake and sleep states. It also studies the role played by antiepileptic drugs (AEDs) on EEG desynchronization in the above cohort. METHODS: A total of 120 PWE divided into 3 groups (each n = 40; juvenile myoclonic epilepsy [JME], temporal lobe epilepsy [TLE]. and extratemporal lobe epilepsy [Ex-TLE]) were subjected to overnight polysomnography. Each patient group was subdivided into drug-naive and on treatment (Each n = 20). EEG phase synchronization analysis was performed to compare peri-IED phase synchronization indices (SI) during awake and sleep stages and between drug naïve and on treatment groups in 4 frequency bands, namely delta, theta, alpha, and beta. The mean ± SD of peri-IED SI among various subgroups was compared employing a multilevel mixed effects modeling approach. RESULTS: Patients with JME had increased peri-IED cortical synchrony in N3 sleep stage, whereas patients with partial epilepsy had increased IED cortical synchrony in N1 sleep stage. On the other hand, peri-IED synchrony was lower during wake and REM sleep. We also found that peri-IED synchronization in patients with JME was higher in drug-naive patients compared with those on sodium valproate monotherapy in theta, alpha, and beta bands. CONCLUSION: The findings of this study suggest that sleep stages can alter cortical synchrony in patients with JME and focal epilepsy, with NREM IEDs being more synchronized and wake/REM IEDs being less synchronized. Furthermore, it also suggests that AEDs alleviate seizures in PWE by inhibiting cortical synchrony.

Heightened Background Cortical Synchrony in Patients With Epilepsy: EEG Phase Synchrony Analysis During Awake and Sleep Stages Using Novel Ensemble Measure.

INTRODUCTION: Excessive cortical synchrony within neural ensembles has been implicated as an important mechanism driving epileptiform activity. The current study measures and compares background electroencephalographic (EEG) phase synchronization in patients having various types of epilepsies and healthy controls during awake and sleep stages. METHODS: A total of 120 patients with epilepsy (PWE) subdivided into 3 groups (juvenile myoclonic epilepsy [JME], temporal lobe epilepsy [TLE], and extra-temporal lobe epilepsy [Ex-TLE]; n = 40 in each group) and 40 healthy controls were subjected to overnight polysomnography. EEG phase synchronization (SI) between the 8 EEG channels was assessed for delta, theta, alpha, sigma, and high beta frequency bands using ensemble measure on 10-second representative time windows and compared between patients and controls and also between awake and sleep stages. Mean ± SD of SI was compared using 2-way analysis of variance followed by pairwise comparison ( P ≤ .05). RESULTS: In both delta and theta bands, the SI was significantly higher in patients with JME, TLE, and Ex-TLE compared with controls, whereas in alpha, sigma, and high beta bands, SI was comparable between the groups. On comparison of SI between sleep stages, delta band: progressive increase in SI from wake ⇒ N1 ⇒ N2 ⇒ N3, whereas REM (rapid eye movement) was comparable to wake; theta band: decreased SI during N2 and increase during N3; alpha band: SI was highest in wake and lower in N1, N2, N3, and REM; and sigma and high beta bands: progressive increase in SI from wake ⇒ N1 ⇒ N2 ⇒ N3; however, sigma band showed lower SI during REM. CONCLUSION: This study found an increased background cortical synchronization in PWE compared with healthy controls in delta and theta bands during wake and sleep. This background hypersynchrony may be an important property of epileptogenic brain circuitry in PWE, which enables them to effortlessly generate a paroxysmal EEG depolarization shift.

Connectivity in ictal single photon emission computed tomography perfusion: a cortico-cortical evoked potential study.

Subtraction ictal and interictal single photon emission computed tomography can demonstrate complex ictal perfusion patterns. Regions with ictal hyperperfusion are suggested to reflect seizure onset and propagation pathways. The significance of ictal hypoperfusion is not well understood. The aim of this study was to verify whether ictal perfusion changes, both hyper- and hypoperfusion, correspond to electrically connected brain networks. A total of 36 subtraction ictal and interictal perfusion studies were analysed in 31 consecutive medically refractory focal epilepsy patients, evaluated by stereo-electroencephalography that demonstrated a single focal onset. Cortico-cortical evoked potential studies were performed after repetitive electrical stimulation of the ictal onset zone. Evoked responses at electrode contacts outside the stimulation site were used as a measure of connectivity. The evoked responses at these electrodes were compared to ictal perfusion values noted at these locations. In 67% of studies, evoked responses were significantly larger in hyperperfused compared to baseline-perfused areas. The majority of hyperperfused contacts also had significantly increased evoked responses relative to pre-stimulus electroencephalogram. In contrast, baseline-perfused and hypoperfused contacts mainly demonstrated non-significant evoked responses. Finally, positive significant correlations (P < 0.05) were found between perfusion scores and evoked responses in 61% of studies. When the stimulated ictal onset area was hyperperfused, 82% of studies demonstrated positive significant correlations. Following stimulation of hyperperfused areas outside seizure onset, positive significant correlations between perfusion changes and evoked responses could be seen, suggesting bidirectional connectivity. We conclude that strong connectivity was demonstrated between the ictal onset zone and hyperperfused regions, while connectivity was weaker in the direction of baseline-perfused or hypoperfused areas. In trying to understand a patient's epilepsy, one should consider the contribution of all hyperperfused regions, as these are likely not random, but represent an electrically connected epileptic network.

Phase Synchronization Analysis of Natural Wake and Sleep States in Healthy Individuals Using a Novel Ensemble Phase Synchronization Measure.

PURPOSE: Electrical activity in the brain is presumed to arise from a combination of tonic asynchronous neuronal firing during wake and a synchronized, burst-pause firing of large number of neurons during sleep. This study aims to compare the phase synchronization index (SI) across multiple channels during wake and various sleep stages on scalp electroencephalographic recordings. METHODS: Forty healthy subjects were subjected to overnight polysomnography using 8-channel electroencephalography. Electroencephalographic phase synchronization during awake, non-rapid eye movement (N1, N2, N3), and rapid eye movement sleep states was studied using ensemble measure (multichannel measure across all the eight channels based on Hilbert transformation between any two pairs). RESULTS: With the progression of states of wakefulness to non-rapid eye movement sleep, there was progressive increase in phase SI in delta band while SI decreased in alpha band (P < 0.001). The SI in delta band during rapid eye movement was comparable with that of awake state (P < 0.001). In theta band, SI tends to decrease in N2 and increase in N3 (P < 0.001). In beta band, there was progressive increase in SI from awake to non-rapid eye movement stages that decreased in rapid eye movement stage (P < 0.001). CONCLUSIONS: This is the first study that has used an ensemble measure to assess the long-range cortical phase synchronization during awake and various sleep stages. The findings support the previous view of increased delta synchrony during non-rapid eye movement sleep and alpha synchrony during wakefulness. Rapid eye movement stage was characterized by marked desynchrony in all frequency bands. These findings suggest the possible role of cortical synchronization in influencing the occurrence of epileptic activity during sleep and awake states.

Effect of carbamazepine on the sleep microstructure of temporal lobe epilepsy patients: a cyclic alternating pattern-based study.

BACKGROUND: Studies looking at the effect of anti-epileptic drugs on the sleep microstructure of patients with epilepsy are scarce. The aim of this study was to compare the sleep microstructural characteristics of drug-naive temporal lobe epilepsy (TLE) patients and those on carbamazepine (CBZ) monotherapy. METHODS: Three age-matched (p = 0.286) and sex-matched (p = 0.398) groups were studied: drug-naive TLE (n = 20); TLE on CBZ (n = 20); and healthy controls (n = 40). All groups underwent overnight polysomnography. Scoring and analysis of arousals and cyclic alternating pattern (CAP) parameters were performed. Comparison of arousal parameters and CAP parameters was performed using either one-way analysis of variance or the Kruskal-Wallis test, followed by pairwise comparisons (p ≤ 0.05). RESULTS: Rapid eye movement (REM) arousal indices and overall CAP rates were higher in patients with TLE (group 1, p < 0.001; group 2, p < 0.001) compared to controls. Furthermore, the overall CAP rate was higher in patients on CBZ. The CAP cycle/sequences indices (group 1, p < 0.001; group 2, p < 0.001) were higher, and conversely, the average duration of CAP cycles/sequences (group 1, p = 0.018; group 2, p = 0.003) was lower in patients with TLE. Finally, an increase in A2 percentage was noted in patients with TLE (group 1, p = 0.011; group 2, p = 0.011). CONCLUSION: We found significant alterations in REM arousal indices and CAP parameters in patients with TLE as compared to controls. Moreover, many of these CAP alterations were greater in patients on CBZ. These findings suggest that anti-epileptic drugs such as CBZ may augment arousal instability in patients with TLE, and hence worsen sleep quality and continuity.

Complications and oncologic outcomes following elective neck dissection with salvage laryngectomy for the N0 neck.

PURPOSE: To investigate the difference in survival and complication outcomes between patients with a clinically and radiologically N0 neck who received an elective neck dissection at the time of salvage total laryngectomy compared to those who had salvage total laryngectomy alone. MATERIALS AND METHODS: A retrospective chart review was performed on 125 salvage total laryngectomy patients who were clinically and radiologically N0 preoperatively. Performance of an elective neck dissection and other factors were tested for associations with various postoperative complications, disease-free survival, and overall survival. RESULTS: Ninety-eight patients underwent elective neck dissection, of which ten had positive nodal pathology. Elective neck dissection was not significantly associated with complications or survival outcomes. Positive nodal disease was associated with worse disease-free and overall survival on multivariate analysis. CONCLUSIONS: In patients with clinically and radiologically N0 necks undergoing salvage total laryngectomy, an elective neck dissection can provide prognostic information but does not appear to be significantly associated with increased complications or improved survival.

Effect of valproate on the sleep microstructure of juvenile myoclonic epilepsy patients - a cross-sectional CAP based study.

OBJECTIVE: Studies looking at the effect of anti-epileptic medications on sleep microstructure of patients with epilepsy are almost non-existent. The aim of this study was to compare sleep microstructural characteristics of drug-naïve juvenile myoclonic epilepsy (JME) patients with those on valproate (VPA) monotherapy. METHODS: Three age- (p = 0.287) and gender- (p = 0.766) matched groups (N = 20 in each group): (1) drug-naïve JME (mean age: 21.2 ± 4.06 years; M : F = 9:11); (2) JME on VPA (mean age: 21.85 ± 4.28 years; M : F = 11:9); (3) healthy controls (mean age: 23.2 ± 3.82 years; M : F = 9:11) underwent overnight polysomnography. Scoring and analysis of arousals American Sleep Disorders Association (ASDA, 2002), cyclic alternating pattern (CAP) (Terzano et al., 2002) parameters were performed. Comparison of arousal and CAP parameters was performed using one-way ANOVA, followed by pairwise comparisons using Fisher's LSD test (p ≤ 0.05). RESULTS: Rapid eye movement (REM) arousal indices were higher in JME patients (Group 1 [p = 0.002] and Group 2 [p <0.001]), whereas the overall and NREM arousal indices were comparable between the three groups. CAP rate was higher in JME patients as compared to controls (p <0.001). Duration of phase A and its subtypes (p <0.001) was reduced in drug-naïve patients as compared to VPA group and controls. Finally, percentage of phase A1 (p = 0.003) was decreased and A3 (p = 0.045) was increased in drug-naïve patients as compared to VPA group and controls. CONCLUSIONS: We found significant alterations in REM arousal indices and several CAP parameters in JME patients. However, many of these alterations were not seen in the valproate group. This might indicate that anti-epileptic medications such as valproate may beneficially modulate arousal instability in JME patients, and hence promote sleep quality and continuity.

Educational Value of a Medical Student-Led Head and Neck Cancer Screening Event.

OBJECTIVE: To evaluate improvement of medical student knowledge of head and neck cancer (HNC) through participation in HNC screening fairs run by medical students. STUDY DESIGN: Prospective cohort study of surveys assessing medical students' knowledge of HNC before and after volunteering at screening fairs. SETTING: Four screening fairs held at the University of Miami Miller School of Medicine during Oral, Head and Neck Cancer Awareness Week. SUBJECTS: Medical student screening fair volunteers. METHODS: Four HNC screening fairs were organized by medical student volunteers. All students completed a preevent survey assessing baseline knowledge and participated in an otolaryngologist-led training session about HNC and the screening examination. During the screening events, students educated guests about HNC and performed physician-guided history and physical examinations. Finally, students completed identical surveys 1 week and 3 months after the event. RESULTS: Thirty-four (n = 34) students completed the preevent surveys. At baseline, 59%, 44%, and 24% named tobacco, alcohol, and human papilloma virus as risk factors, compared with 84%, 81%, and 69% on 3 month follow-up, respectively. Out of 6 analyzed questions, the median total number of correctly answered questions improved from 2 on pretest to 5 at 3 months (P < .0001). CONCLUSION: Volunteer participation in a HNC screening program improves medical students' knowledge of HNC risk factors and symptoms. This innovative approach to students' education via participation and organization of screening events is a useful method of improving their HNC knowledge.

Detection of promoter hypermethylation in salivary rinses as a biomarker for head and neck squamous cell carcinoma surveillance.

PURPOSE: Hypermethylation of tumor suppressor gene promoters has been found in head and neck squamous carcinoma (HNSCC) and other solid tumors. We evaluated these alterations in pretreatment salivary rinses from HNSCC patients by using real-time quantitative methylation-specific PCR (Q-MSP). EXPERIMENTAL DESIGN: Pretreatment saliva DNA samples from HNSCC patients were evaluated for patterns of hypermethylation by using Q-MSP. Target tumor suppressor gene promoter regions were selected based on a previous study describing a screening panel for HNSCC in a high-risk population subjects. The selected genes were: DAPK, DCC, MINT-31, TIMP-3, p16, MGMT, CCNA1. RESULTS: We analyzed the panel in a cohort of 61 HNSCC patients. Thirty-three of the analyzed patients (54.1%) showed methylation of at least one of the selected genes in the saliva DNA. Pretreatment methylated saliva DNA was not significantly associated with tumor site (P = 0.209) nor clinical stage (P = 0.299). However, local disease control and overall survival were significantly lower in patients presenting hypermethylation in saliva rinses (P = 0.010 and P = 0.015, respectively). Multivariate analysis confirmed that this hypermethylation pattern remained as an independent prognostic factor for local recurrence (HR = 12.2; 95% CI = 1.8-80.6; P = 0.010) and overall survival (HR = 2.8; 95% CI = 1.2-6.5; P = 0.016). CONCLUSIONS: We were able to confirm an elevated rate of promoter hypermethylation in HNSCC saliva of patients by using a panel of gene promoters previously described as methylated specifically in HNSCC. Detection of hypermethylation in pretreatment saliva DNA seems to be predictive of local recurrence and overall survival. This finding has potential to influence treatment and surveillance of HNSCC patients.

Evaluation of promoter hypermethylation detection in body fluids as a screening/diagnosis tool for head and neck squamous cell carcinoma.

PURPOSE: To evaluate aberrant promoter hypermethylation of candidate tumor suppressor genes as a means to detect epigenetic alterations specific to solid tumors, including head and neck squamous cell carcinoma (HNSCC). EXPERIMENTAL DESIGN: Using promoter regions identified via a candidate gene and discovery approach, we evaluated the ability of an expanded panel of CpG-rich promoters known to be differentially hypermethylated in HNSCC in detection of promoter hypermethylation in serum and salivary rinses associated with HNSCC. We did preliminary evaluation via quantitative methylation-specific PCR (Q-MSP) using a panel of 21 genes in a limited cohort of patients with HNSCC and normal controls. Using sensitivity and specificity for individual markers as criteria, we selected panels of eight and six genes, respectively, for use in salivary rinse and serum detection and tested these in an expanded cohort including up to 211 patients with HNSCC and 527 normal controls. RESULTS: Marker panels in salivary rinses showed improved detection when compared with single markers, including a panel with 35% sensitivity and 90% specificity and a panel with 85% sensitivity and 30% specificity. A similar pattern was noted in serum panels, including a panel with 84.5% specificity with 50.0% sensitivity and a panel with sensitivity of 81.0% with specificity of 43.5%. We also noted that serum and salivary rinse compartments showed a differential pattern of methylation in normal subjects that influenced the utility of individual markers. CONCLUSIONS: Q-MSP detection of HNSCC in serum and salivary rinses using multiple targets offers improved performance when compared with single markers. Compartment-specific methylation in normal subjects affects the utility of Q-MSP detection strategies.

Positive correlation of tissue inhibitor of metalloproteinase-3 and death-associated protein kinase hypermethylation in head and neck squamous cell carcinoma.

OBJECTIVES/HYPOTHESIS: Promoter hypermethylation of tumor suppressor genes is common in head and neck cancer as well as other primary cancers resulting in epigenetic gene silencing. Tissue inhibitor of metalloproteinase-3 (TIMP-3) has been shown to have promoter hypermethylation in several solid tumors, but has not been identified in head and neck squamous cell carcinoma (HNSCC). Our objective was to determine if TIMP-3 promoter was hypermethylated in HNSCC, if there was any correlation with death associated protein kinase (DAPK), a tumor suppressor whose promoter has been hypermethylated at high levels in HNSCC, and if any clinical factors influence hypermethylation of either of these genes. STUDY DESIGN: Prospective study. METHODS: Tumor samples from 124 patients with HNSCC were evaluated for promoter hypermethylation for TIMP-3 and DAPK using quantitative methylation specific polymerase chain reaction (qMSP). We compared both TIMP-3 and DAPK hypermethylation in HNSCC with each other as well as with other clinical variables. RESULTS: We found that TIMP-3 was hypermethylated in approximately 71.8% of the tumor samples and DAPK was hypermethylated in 74.2%. The presence of TIMP-3 and DAPK promoter hypermethylation was significantly higher than in control specimens. More importantly, TIMP-3 and DAPK hypermethylations in these samples were highly correlated with a concordance of 78% (P < .001). DAPK was also correlated with current alcohol consumption (P < .028), but neither TIMP-3 nor DAPK hypermethylation was significantly correlated with other clinical variables or with survival. CONCLUSION: TIMP-3 promoter hypermethylation is elevated in HNSCC and is highly correlated with DAPK hypermethylation, implying a functional relationship between these genes.

Deleted in colorectal cancer is a putative conditional tumor-suppressor gene inactivated by promoter hypermethylation in head and neck squamous cell carcinoma.

Deleted in colorectal cancer (DCC) is a candidate tumor-suppressor gene located at chromosome 18q21. However, DCC gene was found to have few somatic mutations and the heterozygous mice (DCC(+/-)) showed a similar frequency of tumor formation compared with the wild-type mice (DCC(+/+)). Recently, DCC came back to the spotlight as a better understating of its function and relationship with its ligand (netrin-1) had shown that DCC may act as a conditional tumor-suppressor gene. We evaluated hypermethylation as a mechanism for DCC inactivation in head and neck squamous cell carcinoma (HNSCC). DCC promoter region hypermethylation was found in 75% of primary HNSCC. There was a significant correlation between DCC promoter region hypermethylation and DCC expression (assessed by immunohistochemistry; P = 0.021). DCC nonexpressing HNSCC cell lines JHU-O12 and JHU-O19 with baseline hypermethylation of the DCC promoter were treated with 5-aza-2'-deoxycytidine (a demethylating agent) and reexpression of DCC was noted. Transfection of DCC into DCC-negative HNSCC cell lines resulted in complete abrogation of growth in all cell lines, whereas additional cotransfection of netrin-1 resulted in rescue of DCC-mediated growth inhibition. These results suggest that DCC is a putative conditional tumor-suppressor gene that is epigenetically inactivated by promoter hypermethylation in a majority of HNSCC.

Worldwide distribution of Waardenburg syndrome.

To clarify the multiracial occurrence of Waardenburg syndrome, we present a case series and literature review. A computerized review of the English-language literature was conducted to assess the distribution of reported occurrences of Waardenburg syndrome in populations around the world. We detail the clinical features of 2 family cohorts: one of Western European origin and the other from South Asia. A computerized literature review found sporadic cases of the syndrome in many ethnic groups, including Japanese, Taiwanese, and Middle Eastern families. The highest reported incidence is among Kenyan Africans. Waardenburg syndrome accounts for between 2% and 5% of cases of congenital deafness. It was first described in Northern European cohorts and is widely identified in fair-skinned populations. We hope to raise awareness of the worldwide distribution of this important cause of hearing loss.