Consensus for voice quality assessment in clinical practice: guidelines of the European Laryngological Society and Union of the European Phoniatricians.

INTRODUCTION: To update the European guidelines for the assessment of voice quality (VQ) in clinical practice. METHODS: Nineteen laryngologists-phoniatricians of the European Laryngological Society (ELS) and the Union of the European Phoniatricians (UEP) participated to a modified Delphi process to propose statements about subjective and objective VQ assessments. Two anonymized voting rounds determined a consensus statement to be acceptable when 80% of experts agreed with a rating of at least 3/4. The statements with ≥ 3/4 score by 60-80% of experts were improved and resubmitted to voting until they were validated or rejected. RESULTS: Of the 90 initial statements, 51 were validated after two voting rounds. A multidimensional set of minimal VQ evaluations was proposed and included: baseline VQ anamnesis (e.g., allergy, medical and surgical history, medication, addiction, singing practice, job, and posture), videolaryngostroboscopy (mucosal wave symmetry, amplitude, morphology, and movements), patient-reported VQ assessment (30- or 10-voice handicap index), perception (Grade, Roughness, Breathiness, Asthenia, and Strain), aerodynamics (maximum phonation time), acoustics (Mean F0, Jitter, Shimmer, and noise-to-harmonic ratio), and clinical instruments associated with voice comorbidities (reflux symptom score, reflux sign assessment, eating-assessment tool-10, and dysphagia handicap index). For perception, aerodynamics and acoustics, experts provided guidelines for the methods of measurement. Some additional VQ evaluations are proposed for voice professionals or patients with some laryngeal diseases. CONCLUSION: The ELS-UEP consensus for VQ assessment provides clinical statements for the baseline and pre- to post-treatment evaluations of VQ and to improve collaborative research by adopting common and validated VQ evaluation approach.

Inflammatory complications of vocal fold injection with hyaluronic acid: a multiinstitutional study

Background/aim: This study aimed to assess the inflammatory adverse reactions of vocal fold injection laryngoplasty with hyaluronic acid. Materials and methods: This study was a retrospective chart review of patients who underwent vocal fold injection augmentation with HA injection from January 2005 to September 2016 in nine different institutions. Demographic data, indication for injection, injection techniques, types of injection material, settings of procedure, and complications were reviewed. The types of complication, onset time, and management of complications were also noted. Results: In all, 467 patients were identified. The majority of patients had been injected under general anesthesia (n = 382, 84.7%). For injection material, two different types of hyaluronic acid were used: hyaluronic acid alone or hyaluronic acid with dextranomer. Complications occurred in nine patients (1.9%). The majority of complications were inflammatory reactions (n = 7, 1.47%). Main symptoms were dysphonia and/or dyspnea with an onset of 0 h to 3 weeks after the hyaluronic acid injection. Three patients were hospitalized, one of which was also intubated and observed in the intensive care unit for 24 h. Systemic steroids and antibiotics were the main medical treatment in the majority of cases. There was no statistical difference in complication rates between patients who received hyaluronic acid and those who received hyaluronic acid with dextranomer (P = 0.220). Conclusion: Hyaluronic acid can be considered as a safe substance for the injection of vocal folds with a low risk of inflammatory reaction.

Videolaryngostroboscopy versus microlaryngoscopy for the diagnosis of benign vocal cord lesions: a prospective clinical study.

The aim of this study is to prospectively compare rigid videolaryngostroboscopy with microlaryngoscopy for the diagnosis of benign vocal cord lesions. Eighty-five adult patients with benign vocal cord lesions were evaluated with videolaryngostroboscopy and later underwent microlaryngoscopy. During microlaryngoscopy, systematic examination of the glottis was conducted, including careful inspection and meticulous palpation of the vocal cords from anterior commissure to arytenoids. Preoperative and intraoperative diagnoses were analyzed. One hundred and forty-one lesions were diagnosed preoperatively with rigid videolaryngostroboscopy in 85 patients. Microlaryngoscopy revealed a total of 199 lesions in these patients, demonstrating a 41.1 % higher diagnostic yield. Forty-five (77.6 %) of the 58 additional lesions involved structural abnormalities, including sulcus vocalis, microwebs, vascular ectasia, mucosal bridges, and anterior web. The preoperative diagnosis was consistent with the postoperative diagnosis in only 29 patients (34.2 %). For the rest of the patients (n = 56, 65.8 %), the preoperative diagnosis was either changed, or new lesions were identified during microlaryngoscopy. Intraoperative diagnosis of benign vocal cord lesions differs significantly from preoperative diagnosis, regarding both the type and number of lesions present. A large proportion of patients diagnosed with videolaryngostroboscopy have additional lesions, particularly structural abnormalities. Precise inspection and palpation of vocal cords are thus essential during microlaryngoscopy.

Does Helicobacter pylori exist in vocal fold pathologies and in the interarytenoid region?

The aim of this study was to investigate the existence of Helicobacter pylori (HP) in patients with benign and malignant vocal fold pathologies. This was a prospective clinical study conducted at a tertiary-care academic medical center. Fifty consecutive patients who had undergone microlaryngoscopy between August 2007 and July 2009 were included in the study. The patients with a reflux symptom index (RSI) above 12 and a reflux finding score (RFS) above 6 were accepted as having laryngopharyngeal reflux. Patients with urea breath test (UBT), HP-IgG, and HP cytotoxin-associated gene A (CagA)-IgG positivity were diagnosed as HP positive. During laryngoscopy, two surgical specimens were obtained, one from the primary vocal fold pathology and one from the interarytenoid region. The interarytenoid biopsy specimen was used for HP culture and PCR. The specimen from the vocal fold pathology was used to investigate the presence of HP. RSI was positive in 23 (46%) patients. The RFS positivity was 56%. The presence of HP was confirmed by UBT in 35 (70%), HP-IgG in 37 (74%), and HP CagA-IgG in 38 (76%) patients. There was no difference between RFS-positive and RFS-negative patients in terms of HP-IgG and UBT. None of the interarytenoid or vocal fold specimens showed the presence of HP. HP was not found in the histological specimens of vocal fold pathologies and the interarytenoid region. The presence of HP in the gastric mucosa does not have an effect on the RFS and RSI.

Diagnosis and management of necrotizing fasciitis of the head and neck.

Necrotizing fasciitis is a rapidly progressing and life-threatening soft tissue infection that often affects the abdominal wall, perineum, or extremities following surgery or trauma. It predominantly occurs in elderly and immunocompromised patients. It is rarely seen in the head and neck region. Necrotizing fasciitis of the head and neck carries high rates of morbidity and mortality. Symptoms usually develop quickly and well-timed diagnosis is critical to optimizing outcome. Diagnosis is based on a combination of clinical history, Gram staining and culture, imaging and surgical exploration. Early and aggressive surgical management and urgent parenteral antibiotic therapy are critical to optimizing outcome.

Evaluation of the effectiveness of topical ciprofloxacin and prednisolone in the prevention of myringosclerosis.

An antibacterial drug, ciprofloxacin, has been reported to modulate the inflammatory and immune responses on monocytes. Our objective is to evaluate the effectiveness of ciprofloxacin in the prevention of myringosclerosis that is created by myringotomy in rats and to compare it with prednisolone. In this study, 24 healthy Sprague-Dawley type rats of age 3-4 months and 250-300 g weight were used. Rats have been divided into three random groups. Topical ciprofloxacin was administered to the rats in the first group and topical prednisolone was administered to the rats in the third group; no medication was applied to their opposite ears and they were determined as control group. Prednisolone was administered to one ear of the rats and ciprofloxacin was administered to their opposite sides in the second group. After bilateral myringotomies the treatments were applied for 20 days as five drops two times a day. Otomicroscopic examination was made on the 10th day and reperforations were made when necessary. Rats were killed on the 21st day and temporal bone dissections were done. When we evaluate in terms of myringosclerosis and tympanic membrane thickness, in the first group, myringosclerosis and thickness have been observed in two of seven ears (28.6%) where ciprofloxacin was administered and in five of the seven ears (71.4%) where no treatment was applied. In the second group where prednisolone and ciprofloxacin were compared, myringosclerosis and thickness have been observed in six of the ten ears (60.0%) where prednisolone was administered and in four of the ten ears (40.0%) where ciprofloxacin was administered. In the third group, myringosclerosis and thickness have been observed in three of seven ears (42.9%) where prednisolone was administered and in four of the seven ears (57.1%) where no treatment was applied. All histopathological evaluations were made by one pathologist in a blinded manner. In our study, the effect of the ciprofloxacin and prednisolone was similar in preventing the experimental myringosclerosis and TM thickness in rats. When compared with the control groups, this preventive effect was more obvious in the ciprofloxacin treatment group than that of prednisolone.

Reflux Symptom Index: Translation to the Kazakh Language and Validation.

BACKGROUND: This research focuses on the translation and validation of the Reflux Symptom Index (RSI) in Kazakh language and the comparison of its results among patients with symptoms of Laryngopharyngeal reflux (LPR) and without LPR symptoms. OBJECTIVE: This study aims to translate and validate the RSI in Kazakh language (Kz-RSI). METHODS: The RSI from the original American English version has been translated according to standard procedures into Kazakh language. The study included 248 patients, 124 LPR patients, and 124 asymptomatic controls. A final version of the Kz-RSI was tested twice: on Day 0 and Day 14 for participants without LPR symptoms and on Day 0 and posttreatment for those with LPR symptoms. Reliability, validity, and internal consistency of test-retest were calculated. RESULTS: The Cronbach's alpha value for Kz-RSI was 0.94, indicating excellent internal consistency. The single measurement absolute agreement of the interclass correlation coefficient was 0.90 (95% confidence interval [0.86; 0.93]), which indicates good retest reliability. The total Kz-RSI scores and each item were significantly higher in the LPR group than in the control group (P < 0.001). In the posttreatment LPR patient group, the Kz-RSI scores for each item and overall were significantly lower than the pretreatment scores (P < 0.001). CONCLUSIONS: Kz-RSI has been successfully translated and validated to identify LPR among the Kazakh-speaking population. Moreover, it is an inexpensive method with strong internal consistency, high retesting rates, reliability, and optimal clinical validity.

Laryngeal Inflammatory Myofibroblastic Tumor With Anemia and Thrombocytosis.

Inflammatory myofibroblastic tumor (IMT) of the larynx is a rare benign lesion that commonly occurs in the soft tissues. We present the first case with systemic manifestations of laryngeal IMT that was associated with hypochromic, microcytic-type anemia and thrombocytosis.

Association Between Nasal Obstruction Symptoms and Anxiety.

AIM: The aim of this study was to determine the correlation between nasal function assessment and anxiety scales. METHODS: A total of 120 patients with the complaint of nasal obstruction were classified as nasal septum deviation group (DNS) and no nasal pathology group (NON). A control group was formed of 57 healthy participants. Nasal obstruction severity was assessed using the Nasal Obstruction Symptom Evaluation (NOSE) scale, nasal resistance level with rhinomanometry and anxiety levels with the Agoraphobic Cognitions Questionnaire (ACQ), and the Body Sensations Questionnaire (BSQ). RESULTS: There was a statistically significant difference between both the DNS and the NON groups and control group in terms of NOSE scale, ACQ, and BSQ (P < .001). The total nasal resistance values were higher in the DNS group compared to both the NON and control groups (P < .001), although the difference between the NON group and control group was not statistically significant. CONCLUSION: The results of this study showed that patients with nasal breathing complaints, but no organic pathology, had the same level of nasal obstruction symptoms as patients with nasal septal deviations. Anxiety levels are elevated in patients with symptoms of nasal obstruction, even when there is lack of organic nasal pathology.

Laryngoscopic Examination During the COVID-19 Pandemic: Turkish Voice Speech and Swallowing Disorders Society and Turkish Professional Voice Society Recommendations.

COVID-19 is highly transmissible and spreads rapidly in the population. This increases the occupational risk for health care workers. In otolaryngology clinic practice, patients with upper respiratory tract infection symptoms are common. Also, routine head and neck examinations such as oral cavity examination, nasal/nasopharyngeal examination, or video laryngostroboscopic evaluation are highly risky because of the aerosol formation. To emphasize this issue, two leading otolaryngology organizations in Turkey; 'Voice Speech and Swallowing Disorders Society', and 'Professional Voice Society' gathered a task force. This task force aimed to prepare a consensus report that would provide practical recommendations of the safety measurements during routine clinical care of laryngology patients. To fulfill this, universal aim, on the 2nd and 9th of May 2020, two web-based meetings were conducted by 20 expert physicians. This eighteen items list was prepared as an output.

[Repair of nasal septal perforation with different intranasal flap techniques and their outcomes]. / Nazal septal perforasyonun farkli intranazal flep teknikleri ile onarimi ve sonuçlari.

OBJECTIVES: The aim of this study was to reveal the efficacy of different nasal mucosal flap techniques in the repair of nasal septal perforation. PATIENTS AND METHODS: Between April 2006 and May 2009 21 patients (12 males, 9 females; mean age 36.6+/-12.7 years; range 17 to 60 years) with the complaints of nasal obstruction, bleeding, crusting, whistling during inspiration, and pain and in whom septum perforation was detected were operated on and they were included in this study. The patients were followed-up for an average of 16.9 months (3 to 35 months). Cross-stealing technique was performed on 11 patients while advancement flap was performed on three patients and rotation flap was performed on seven patients. Patients were followed-up for at least three months before the evaluation of the postoperative results. RESULTS: Complete closure was observed in 16 out of 21 patients (76.2%) and partial closure in one patient (4.8%). In four patients (19%) perforation was not closed and its size remained unchanged. CONCLUSION: In the literature, many different surgical techniques have been described for the repair of nasal septal perforation. The main aim of the repair is not only the closure of perforation but also the restoration of normal function and physiology in the nose. In order to achieve this, the most physiologically and anatomically suitable method is the closure of nasal septal perforation with three layers composed of two mucoperichondrial flaps and one interpositional graft. Although cross-stealing technique may be an anatomically and physiologically feasible option for the closure of small-middle sized perforation located anteriorly, the highest success rates are obtained with advancement and rotation flaps when the location and size of perforation are considered.

[Internal jugular vein thrombosis following functional and selective neck dissections]. / Fonksiyonel ve selektif boyun diseksiyonu sonrasi internal juguler ven trombozu.

OBJECTIVES: Internal jugular vein (IJV) thrombosis is a rare complication of functional and selective neck dissections. It increases morbidity and may seldom be fatal. We investigated the frequency of IJV thrombosis and its relationship with the dissection technique. PATIENTS AND METHODS: We evaluated 52 functional and selective neck dissections performed in 34 male patients (mean age 57 years; range 34 to 76 years) with head and neck cancer. Dissections were mainly performed by sharp dissection (n=27) or cautery (n=25). The patients were examined by Doppler ultrasonography with respect to IJV flow and thrombosis preoperatively, and at two weeks and at 3 to 6 months postoperatively. RESULTS: In the early postoperative period, thrombosis was observed in 7.4% (n=2) of the necks treated with sharp dissection and in 4% (n=1) of the necks treated with cautery. There was no statistically significant difference between the two groups with respect to IJV thrombosis. Late Doppler examinations showed complete recanalization of all thrombosed IJVs. CONCLUSION: Our data suggest that sharp dissection or cautery techniques performed in functional neck dissections do not differ with respect to the frequency of postoperative IJV thrombosis.

[Reliability and validity of the Turkish version of the Voice Handicap Index]. / Ses Handikap Endeksi (Voice Handicap Index) Türkçe versiyonunun güvenilirligi ve geçerliligi.

OBJECTIVES: We investigated the reliability and validity of the Turkish version of the Voice Handicap Index (VHI), and developed a short VHI form that would be more practical. PATIENTS AND METHODS: The original VHI was translated to Turkish by 10 otolaryngologists, then it was translated back to English by a linguist, and the final text was prepared by the evaluation committee composed of three members. The translated version was administered to a group of 220 subjects twice with 7-14 days intervals. Based on the responses, statistical analyses were performed to assess its reliability and validity. RESULTS: Internal consistency reliability was found to be highly significant (Cronbach's alpha=0.97). Test-retest correlation coefficient was 0.93 for the total score. Factor analysis yielded three factors explaining 64.8% of the total variance. The corrected item-total correlation coefficients ranged from 0.50 to 0.80. The 10 most robust VHI items, namely, E7, E9, P10, F11, F12, E15, F16, P18, P20 and E29, were selected using the corrected item-total correlation coefficients, and a shortened form of the Turkish VHI was developed. CONCLUSION: As some items are thought to be contentious in the Turkish VHI, the short form of the Turkish VHI is more suitable for use in clinics.

Intratympanic methylprednisolone for sudden sensorineural hearing loss.

OBJECTIVE: Corticosteroids are commonly used for the treatment of sudden sensorineural hearing loss (SSHL). In this study, the effectiveness of intratympanic (IT) corticosteroid injection was studied and compared with a control group on patients with SSHL who failed systemic corticosteroid treatment. MATERIALS AND METHODS: A total of 19 patients as a retreatment group (RG) and 18 patients as a control group (CG), all failed high-dose intravenous and oral corticosteroid treatments, were included in this study. These patients were invited back, and IT methylprednisolone was injected five times via 3-day intervals in RG and followed-up for a mean period of 24.9 months (range, 7-30 mo). Audiological evaluations were performed initially, a week after the completion of the injections, monthly in the following first 3 months, and at the end of follow-up period in RG. The CG was followed-up for 3 months after the completion of systemic corticosteroid treatment without any additional drug administration. RESULTS: The mean age was 52.6 years (range, 20-79 yr) in RG and 59.9 years in CG. The mean pure-tone average for speech frequencies (500-4,000 Hz) at baseline audiogram and at the first month, at the third month, and at last controls were 65.2 (range, 43-102 dB), 45.4 (range, 23-77 dB), 43.6 (range, 30-77 dB), and 44.5 (range, 33-77 dB) dB, respectively, in RG. The mean pure-tone averages for speech frequencies (500-4,000 Hz) at the end of systemic treatment and at third-month control were 63.5 (range, 44-98 dB) and 59.0 (range, 40-100 dB) dB, respectively, in CG. The hearing gain that is equal to or more than 10 dB was achieved in 14 patients (73.6%) at the last control in RG. No hearing gain could be detected in the CG. No serious side effect was observed with IT treatment. CONCLUSION: We conclude that IT methylprednisolone injection provides more significant hearing improvement for patients that failed with previous high-dose systemic corticosteroid administration than systemic corticosteroid treatment alone. So it may be the first-step medical treatment of idiopathic SSHL alone or at least may be combined with the systemic corticosteroid administration.

Acoustic analysis findings in objective laryngopharyngeal reflux patients.

OBJECTIVE: The aim of this study was to identify the effects of objective laryngopharyngeal reflux (LPR) on the acoustic parameters of patients by comparing their voice samples with that of control subjects. STUDY DESIGN: Prospective study in two tertiary reference hospitals. METHODS: 48 consecutive patients with symptoms related to LPR and 64 control subjects were included in the study. Suspected LPR patients underwent a 24-hour ambulatory pH monitoring, and 25 (52%) of them were shown to have objective LPR. Acoustical evaluation results of objective LPR patients were compared with that of symptomatic LPR patients and control subjects. RESULTS: All frequency perturbation values obtained from objective and symptomatic LPR patients were higher than the control subjects (P<0.01). Mean fundamental frequency, amplitude perturbation measures, and noise-to-harmonics ratio were not significantly different between groups. CONCLUSION: LPR patients have significantly different frequency perturbation values than control subjects.

Familial primary localized laryngeal amyloidosis in two sisters.

Laryngeal amyloidosis is rare, accounting for less than 1% of all benign laryngeal tumors. Although familial primary localized amyloidosis has been reported in other parts of the body, no familial cases have been reported in the larynx. Primary localized laryngeal amyloidosis was detected in two sisters whose ages were 35 years and 38 years, respectively. In the elder patient, a previous endolaryngeal biopsy for symptoms of dysphonia yielded no pathologic findings. Laryngoscopic examination of the patient showed a significant submucosal accumulation at the level of ventricles and vocal folds. The younger sister had a complaint of hoarseness for five years. The results of endolaryngeal biopsies performed in both patients were reported as amyloidosis. Further evaluations were negative for systemic amyloidosis. No surgical intervention was considered. The patients were monitored for more than two years without any other coexisting disease.

Pediatric Vocal Fold Nodule Etiology: What Are Its Usual Causes in Children?

OBJECTIVES: In this study, the relation between phonotrauma and presence of siblings and social activities was investigated, and the incidence of voice disorders in the mothers of children with vocal fold nodules was studied with objective (clinical voice analysis) and subjective (laryngostroboscopy, Voice Handicap Index) methods. METHODS: Twenty-nine children with vocal fold nodules (age range 5-14 years), 45 age-matched children without any voice disorders as a control group, and their mothers were included in the study. All patients had laryngostroboscopy and clinical voice analysis, and their mothers filled out the Pediatric Voice Handicap Index. We noted the most common place or situation where children used their voice in excessively high volume according to their mothers, including home, school, sportive activities, and singing or reciting poem activities, to recognize the major cause of phonotrauma. In addition, presence of siblings was recorded. RESULTS: It was found that 15 patients (51.7%) had younger siblings, seven patients (24.1%) had older siblings, five patients (17.2%) had both younger and older siblings, and two patients (6.8%) did not have any siblings. It was seen that excessive usage of high-volume voice at home had a correlation with presence of only younger siblings, and both younger and older siblings tended to cause phonotrauma at home (86.7%). Additionally, eight boys (44.4%) reported presence of sportive activities, whereas none of the girls had such an activity (P = 0.012). CONCLUSION: Presence of siblings seems to be an important factor for vocal nodule formation. Maternal relationship does not seem to be a major factor for vocal misuse.

[Validation and reliability of Turkish Singing Voice Handicap index]. / Özgün San Sesi Handikap Endeksi ölçeginin Türkçe versiyonunun geçerlik ve güvenirligi.

OBJECTIVES: This study aims to constitute a valid and reliable Turkish version of the original Singing Voice Handicap Index. PATIENTS AND METHODS: An authorized committee assessed the reliability and validity of the content, scope, and language of the original Singing Voice Handicap Index which underwent a back translation process. The Turkish version of the questionnaire was answered twice with a 7 to 10-day interval by two singing voice groups with or without singing voice problems. The reliability and validity analyses were performed based on these answers. RESULTS: Of a total of 123 individuals (64 females, 59 males; mean age 26.2±7.3 years), 81 were without a voice pathology and 42 were with a voice pathology. The total Cronbach's alpha coefficient was 0.917. The item-total correlations ranged between 0.51 and 0.89. The weighted kappa values of test-retest correlation values of the items were 0.82-0.91. The Cronbach's alpha values of two part of the questionnaire based on the split-half method were 0.89 and 0.84. The mean total scale scores were 21.8±18.5 and 53.6±28.9 in normal and pathology groups, respectively and there was a statistically significant difference in scores between these two groups (p=0.000). CONCLUSION: The Turkish version of the Singing Voice Handicap Index is a valid and reliable scale which can be used in the evaluation of voice problems of Turkish-speaking singing voice users.

Evaluation of sentinel nodes in the assessment of cervical metastases from head and neck squamous cell carcinomas.

AIMS AND BACKGROUND: To delineate the lymphatic drainage in head and neck squamous cell carcinoma (SCC) patients by lymphoscintigraphy and to investigate whether the results of histopathological evaluation of sentinel nodes have any diagnostic relevance with regard to the metastatic involvement of all cervical lymph nodes. METHODS: Forty-one patients clinically pre-diagnosed as having primary head and neck SCC underwent Tc-99m nanocolloid lymphoscintigraphy and were evaluated according to the Memorial Sloan Kettering cervical lymph nodes level system. Treatment of 24 of these patients included neck dissection in addition to surgery for the primary tumor. RESULTS: Among the 24 operated patients nine were found to be pathologically N+. In two (22%) of these nine patients there were histopathological metastases in non-sentinel lymph nodes. In one patient with a metastatic lymph node, no sentinel nodes were identified. Altogether, lymphoscintigraphic sentinel node detection failed in three (33%) cases. CONCLUSION: The procedure of lymphoscintigraphic imaging of sentinel lymph nodes and its use in the determination of squamous cell carcinoma metastases is theoretically promising. However, in order to assess the true value of sentinel lymph node imaging and make treatment plans based on this technique, it's necessary to increase the number of cases included in the studies, and obtain results that are specific for the primary tumor and involved lymph nodes.

The carpenter syndrome phenotype.

Carpenter syndrome (Acrocephalopolysyndactyly type II), first described in 1901, consists of acrocephaly, syndactyly, polydactyly, congenital heart disease, mental retardation, hypogenitalism, cryptorchidism, obesity, umbilical hernia and bony abnormalities. We report a 6 years old boy presenting as a union of these malformations and also having bilateral sensorineural hearing loss. Auditory disturbances are not common among Carpenter syndrome patients. According to our knowledge, this is the first Carpenter syndrome case whose hearing loss is demonstrated by auditory brainstem response (ABR) test.