A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent / 대한이비인후과학회지

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.

A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent / 대한이비인후과학회지

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.

Short-Term Clinical Outcome of Neuro One® System Cochlear Implantation in Korean Adults / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study analyzed short-term clinical outcome and safety of Neuro One® (Oticon Medical) cochlear implantation on postlingually deafened Korean adults. SUBJECTS AND METHOD: A prospective study was performed on five postlingually deafened Korean adults who underwent Neuro One® cochlear implantation between April and June, 2016. All cases were successfully implanted via round window approach. Preoperative pure tone audiometry and speech perception score [phonetically balanced (PB) words, bi-syllable words, Korean version-central institute for the deaf (K-CID)], intraoperative evoked compound action potential (ECAP), postoperative audiometry, aided warble-tone thresholds and speech perception scores after 6 months were evaluated. Residual hearing group was defined as patients with below 40 dB of average audiometric threshold in low frequency (250 and 500 Hz). Preservation of residual hearing and postoperative complication were also evaluated. RESULTS: The mean age of five patients (3 men, 2 women) was 37.6 years. All of the recipients were unsatisfied about the conventional hearing aid. Two patients met the criteria for belonging to a residual hearing group. The audiometric threshold on the implanted side was 102.8 dB, and the opposite side was 89.6 dB. The speech perception score with hearing aid resulted in the PB word score of 35.5%, bi-syllable word score of 50%, and the open set sentence test of 62.5% on the average. The mean operation time was 87 minutes. Three patients showed normal ECAP response throughout the entire electrode intraoperatively, but the other two patients could not detect ECAP response intraoperatively. However, we could eventually identify ECAP responses in all cases postoperatively. All patients of residual hearing group could preserve their residual hearing without deteriorating more than 10 dB on audiometry. At 6 months after implantation, there was significant improvement of speech perception scores (PB word score was 54.1%; bi-syllable word score was 68.1% and K-CID was 86.2%). CONCLUSION: Neuro One® cochlear implant system was able to preserve residual hearing with relative short operation time. Short-term clinical outcome showed no significant difference with other cochlear implant devices. However, intraoperative ECAP measurement was not consistent. Further investigation was necessary.

Comparison of Video Head Impulse Test with Posterior Semicircular Canal Plane and Cervical Vestibular Evoked Myogenic Potential / 대한평형의학회지

OBJECTIVE: Cervical vestibular evoked myogenic potential (cVEMP) is thought to be assessing the function of the saccule and inferior vestibular nerve. Therefore, cVEMP indirectly reflects the function of the posterior semicircular canal. Recently, the video head impulse test (vHIT) is considered as useful clinical tool to detect each semicircular canal dysfunction. Goal of this study was to evaluate and compare the results of cVEMP with posterior canal plane of vHIT (p-vHIT). METHODS: Retrospectively, we compared the results of cVEMP with p-vHIT in 43 patients who visited with dizziness. We analyzed the inter-test agreement of cVEMP with p-vHIT. RESULTS: Positive asymmetry of cVEMP was present in 37.2% (16/43), and no responses of both ears were identified in 16.3% (7/43). In p-vHIT analysis, unilateral positive was 27.9% (12/43), bilateral positive was 11.6% (5/43) and negative in both sides was 60.5% (26/43). The inter-test agreement between cVEMP and p-vHIT was 75.8% (25/33) as we considered even in lesion side. And, Fleiss's kappa value showed a fair to good agreement (kappa value=0.559). In bilateral no response group (7 patients) in cVEMP, variable additional information could be obtained using p-vHIT. CONCLUSION: cVEMP and p-vHIT showed relatively lower inter-test agreement than expected. But, p-vHIT could be easily performed, and give additional information for differential diagnosis.

A Case of Perilymphatic Fistula Misdiagnosed as Benign Paroxysmal Positional Vertigo / 대한이비인후과학회지

Perilymphatic fistula (PLF) is an uncommon disease characterized by abnormal leakage of the perilymph from the inner ear into the middle ear. The symptoms of PLF include sudden-onset hearing loss, tinnitus, ear fullness, and vertigo resulting from inner-ear trauma, stapedial surgery or barotrauma such as valsalva, and nose blowing. As nystagmus can be induced by the affected-ear in down position, benign positional paroxysmal vertigo (BPPV) should be considered for differential diagnosis. About 40-50% of PLF patients have experienced spontaneous healing, but surgical repair should be considered when the hearing symptom and dizziness are not alleviated or are aggravated within a few days. Reported herein, with a review of the relevant literature, is a case of PLF initially misdiagnosed as BPPV but where successful fistula repair was finally achieved.

In Reply: Dominance of Ossicular Route in Sound Transmission

No abstract available.

Long-term Outcome of Cochlear Implant in Patients with Chronic Otitis Media: One-stage Surgery Is Equivalent to Two-stage Surgery

This study compared long-term speech performance after cochlear implantation (CI) between surgical strategies in patients with chronic otitis media (COM). Thirty patients with available open-set sentence scores measured more than 2 yr postoperatively were included: 17 who received one-stage surgeries (One-stage group), and the other 13 underwent two-stage surgeries (Two-stage group). Preoperative inflammatory status, intraoperative procedures, postoperative outcomes were compared. Among 17 patients in One-stage group, 12 underwent CI accompanied with the eradication of inflammation; CI without eradicating inflammation was performed on 3 patients; 2 underwent CIs via the transcanal approach. Thirteen patients in Two-stage group received the complete eradication of inflammation as first-stage surgery, and CI was performed as second-stage surgery after a mean interval of 8.2 months. Additional control of inflammation was performed in 2 patients at second-stage surgery for cavity problem and cholesteatoma, respectively. There were 2 cases of electrode exposure as postoperative complication in the two-stage group; new electrode arrays were inserted and covered by local flaps. The open-set sentence scores of Two-stage group were not significantly higher than those of One-stage group at 1, 2, 3, and 5 yr postoperatively. Postoperative long-term speech performance is equivalent when either of two surgical strategies is used to treat appropriately selected candidates.

Erratum: Determinants of Conductive Hearing Loss in Tympanic Membrane Perforation: Correction of the Sixth Author Name

The sixth author's name should have been given as Myung-Whan Suh.

Determinants of Conductive Hearing Loss in Tympanic Membrane Perforation

OBJECTIVES: Tympanic membrane perforations are common, but there have been few studies of the factors determining the extent of the resulting conductive hearing loss. The aims of this study were to determine whether the size of tympanic membrane perforation, pneumatization of middle ear & mastoid cavity, and location of perforation were correlated with air-bone gap (ABG) of patients. METHODS: Forty-two patients who underwent tympanoplasty type I or myringoplasty were included and preoperative audiometry were analyzed. Digital image processing was applied in computed tomography for the estimation of middle ear & mastoid pneumatization volume and tympanic membrane photograph for the evaluation of perforation size and location. RESULTS: Preoperative mean ABG increased with perforation size (P=0.018), and correlated inversely with the middle ear & mastoid volume (P=0.005). However, perforations in anterior versus posterior locations showed no significant differences in mean ABG (P=0.924). CONCLUSION: The degree of conductive hearing loss resulting from a tympanic membrane perforation would be expected with the size of perforation and pneumatization of middle ear and mastoid.

Preoperative Vestibular Function in Adults with Cochlear Implantation: Comparison between Prelingual and Poslingual Deafness / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Cochlear system and vestibular system have close relationship anatomically and developmentally. According to previous literatures, there are high incidences of vestibular dysfunction in subjects with severe hearing loss. The aim of this study is to validate the status of vestibular function in adults with profound hearing loss, and to compare the status of vestibular function between pre-lingual and post-lingual deaf. SUBJECTS AND METHOD: Vestibular function of 59 patients who had profound hearing loss was reviewed retrospectively. Patient's information and audiometry, Korean version Central Institute for the Deaf (KCID) scores, caloric test, rotation chair test and vestibular evoked myogenic potential (VEMP) were analyzed. The subjects were divided into two groups, prelingual deaf (pre-LD, n=12) and postlingual deaf (post-LD, n=47). We analyzed the difference between two groups. RESULTS: Fifty nine point six percent of patients showed hypo-function in caloric test. In the rotational chair test, abnormality of step velocity gain (43.1%), time constant (51.8%), sinusoidal harmonic acceleration (SHA)-gain (53.4%), SHA-phase lead (29.1%) and SHA-asymmetry (23.6%) were found. There was unilateral (20.0%) and bilateral (37.5%) hypo-function when VEMP was tested. Between pre-LD and post-LD groups, VEMP test revealed statistically significant difference between two groups (p=0.020). Post-LD groups had more patients of unilateral or bilateral hypofunction in VEMP test. The KCID score showed no significant correlation with vestibular functions. CONCLUSION: The patients of bilateral profound hearing loss showed unilateral or bilateral vestibular functional abnormality despite of no dizziness. Prevalence of lateral canal dysfunction does not seem to be different between pre-LD group and post-LD group. And post-LD groups have higher probability of saccular dysfunction than pre-LD.

Subjective and Audiologic Results of Bone Anchored Hearing Aids (BAHA) / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study aims to evaluate audiologic results and subjective satisfaction of bone anchored hearing aids (BAHA) patients. SUBJECTS AND METHOD: Preoperative air-conduction and bone-conduction thresholds, postoperative aided thresholds were measured for 14 patients. To evaluate patient satisfaction, two questionnaires derived from single sided deafness (SSD) questionnaire and Bern Benefit in SSD questionnaire were used. RESULTS: The mean age of patients, consisting of 8 males and 6 females, was 40.0+/-5.9 year. Ten patients (71%) received implantations for conductive or mixed hearing loss and 4 (29%) for SSD. The mean follow-up period was 27.8+/-5.6 months. The mean preoperative bone-conduction threshold of conductive or mixed hearing loss group was 21.4 dB and the mean air-conduction threshold was 65.4 dB. The mean bone-conduction threshold of SSD group was 60.0 dB and the mean air-conduction threshold was 105.9 dB. The postoperative aided threshold was significantly improved in conductive or mixed hearing loss group (p=0.005). The patients in the conductive or mixed hearing loss group showed more satisfaction with BAHA than those in the SSD group did, but the degree of satisfaction was not statistically significant (p=0.08 for questionnaire 1, p=0.12 for questionnaire 2). Patients with better preoperative bone-conduction threshold and patients with better preoperative speech discrimination score showed more satisfaction with BAHA in the questionnaire 1 (p=0.045, p=0.036). CONCLUSION: BAHA can be considered effective and beneficial for patients with conductive or mixed hearing loss and SSD. Further studies will be needed to choose the appropriate indications for BAHA, especially in SSD group in Korea.

Audiological and Clinical Finding of Eosinophilic Otitis Media in Korea / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Eosinophilic otitis media (EOM) is characterized by the presence of a highly viscous effusion containing eosinophils. It mainly occurs in patients with bronchial asthma, nasal polyps and is resistant to conventional treatments for otitis media. In these patients, steroid is very effective in controlling the disease. However, the major complication is sensorineural hearing loss, especially at high frequencies, which may occur despite steroid therapy. SUBJECTS AND METHOD: Here we report 10 cases of EOM at Seoul National University Hospital. Clinical courses and characteristics of the patients were analyzed. We compared the hearing deterioration and other clinical variables between EOM patients and age-matched non-EOM chronic otitis media patients. RESULTS: All cases had viscous effusion and 9 cases were associated with asthma and nasal polyps. All patients had a decreased hearing in high frequency range compared to the age matched controls. The average bone conduction threshold difference at 2 kHz, 4 kHz was 22.4 dB HL and 42.5 dB HL, respectively. Among the patients, one showed profound sensorineural hearing loss bilaterally after the onset of EOM and received cochlear implantation. The open set sentence score was 82% 6 months after cochlear implantation. Most of the cases were resistant to conventional treatments for otitis media, such as administration of antibiotic agents, ventilation tube insertion or mastoidectomy. However, cases that were given steroid treatment had improvement in ear symptoms. CONCLUSION: Since EOM patients show deterioration of hearing, it is important to properly diagnose EOM, start adequate treatment and explain to patients that the disease may last for a long period and progressive hearing loss may occur.

Comparison Analysis between Korean Central Institute for the Deaf Sentence and Korean Hearing in Noise Test Sentence / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Hearing in Noise Test (HINT) sentences and Central Institute for the Deaf (CID) sentences are tools that are used for assessing speech cognition. The Korean versions of HINT and CID, KHINT and KCID, have been made and used for the selection of cochlear implant candidates. In this study, we analyzed the compatibility of sentences of KCID and KHINT as testing material for assessing speech performance. MATERIALS AND METHODS: A total of 36 patients selected for the study were divided into 2 groups. Group A, whose mean age was 49.9 years, was composed of 18 patients with hearing aid (range, 24 to 68 years); Group B, whose mean age was 23.5 years, was composed of 18 patients with cochlear implant (range, 10 to 63 years). Open set recognition scores of 2 sentences were compared in each group, by randomly selecting one list each of the KCID and KHINT sentences. Sentences were stimulated using recorded voice and live voice for Group A and B, respectively. RESULTS: There were no statistically significant differences between mean scores of KCID and KHINT sentences in each group (p=0.289, 0.208), respectively. However, there were statistically significant linear correlations between KCID and KHINT sentences in each group and total group (R2=0.961, 0.945, 0.923, respectively; p<0.01, <0.01, <0.01, respectively). CONCLUSION: KCID sentences can be used as a speech performance test material and might serve as a compatible test material for selection of the cochlear implant candidacy.

Recurrent Meningitis in a Patient with Inner Ear Anomaly after Cochlear Implantation / 대한이비인후과학회지

Cochlear implantation (CI) has been regarded as a safe and effective treatment for patients with severe to profound sensorineural hearing loss. The increase of experience in CI thus has had the effect of leading more children with inner ear anomaly to be considered as cochlear implant candidates. For patients with inner ear anomaly, auditory rehabilitation has been reported to be improved after CI; however, there are several factors such as cerebrospinal fluid leakage, facial nerve injury and abnormal positioning of electrode during surgery and uncertainty of auditory rehabilitation and possibility of the recurrent meningitis after CI that should be considered. Several studies proved that the incidence of otogenic meningitis is higher in patients with inner ear anomaly than in patients with normal inner ear anatomy. We experienced a case of recurrent meningitis due to an unusual cause after cochlear implantation in a patient with inner ear anomaly.

Audiologic Results of Ossiculoplasty Using Malleus Footplate Assembly: The Comparison between Autologous Incus and Hydroxyapatite

BACKGROUND AND OBJECTIVES: Absence of incus is one of the most common ossicular anomalies and also frequently found in chronic otitis media or chornic otitis media with cholesteatoma. Ossiculoplasty with malleus footplate assembly (MFA) can be an excellent option in the above situations. We reviewed our procedures and evaluated the hearing results of ossiculoplasty using the MFA. SUBJECTS AND METHODS: This study retrospectively reviews a series of 32 patients who underwent MFA ossiculoplasty between September 2001 and March 2010 in an academic tertiary referral center. The autologous incus or hydroxyapatite (HA) was used as materials for reconstruction. RESULTS: Thirteen of 32 patients (40.6%) had ossicular chain anomaly, 9 patients (28.1%) had congenital cholesteatoma and 8 patients (25.0%) had chronic otitis media with cholesteatoma and 2 patients (6.3%) had chronic otitis media. The patients with ossicular chain ano-maly showed the greatest air-bone gap (ABG) closure. Overall, postoperative ABG was 17.7+/-11.7 dB at postoperative 6 months and 19.7+/-14.3 dB at postoperative 1 year, compared with preoperative ABG of 37.9+/-9.3 dB. There was no statistical difference of ABG at postoperative 12 months, 24 months between MFA using incus (n=17) and HA (n=15)(p=0.300, and p=0.563). There was no meaningful difference of postoperative ABG between the patients with combined mastoidectomy (n=10) and those without mastoidecmoy (n=22)(p=0.555). No extrusions occurred. CONCLUSIONS: MFA can be an effective option in the case of absent incus expecting good postoperative hearing results.

Hearing Rehabilitation with Bone Anchored Hearing Aid: Experience in 14 Patients / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Bone anchored hearing aid (BAHA) is an alternative method applicable to patients with chronic draining ear, congenital aural atresia and single sided deafness, who cannot benefit from conventional air conduction hearing aids. The objective of this study was to evaluate the experience of 14 patients who underwent BAHA surgery. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 14 patients who underwent BAHA surgery. Preoperative pure tone air and bone conduction thresholds and air-bone gap, postoperative BAHA-aided thresholds were measured. Hearing improvement as a result of implantation and complications related to implant were evaluated. RESULTS: The most common indication for BAHA was congenital aural atresia (8 patients) and the rest consisted of chronic otitis media (3 patients) and unilateral sensorineural hearing loss (3 patients). The average threshold improvement with BAHA was 40 dB and 34 dB in patients with congenital aural atresia and chronic otitis media, respectively. Patients with unilateral hearing loss had a postoperative aided threshold of 25 dB. Complications were limited to the periabutment skin problem in two patients. One patient received revision surgery replacing the diseased skin with split-thickness skin graft from a thigh and the other patient received surgery to remove the abutment and the wound was closed with rotation flap, leaving the fixture underneath the skin. CONCLUSION: BAHA could be one of the safe and reliable treatment options available for auditory rehabilitation. Systematic evaluation for candidate selection might be needed to increase hearing gain and decrease co-morbidity.

Correlation between Dose of Ethacrynic Acid and Weight of Cat in the Cat Deafening Procedure / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Co-administration of kanamycin (KM) with the loop diuretic ethacrynic acid (EA) has been known to produce a rapid and profound hearing loss in adult animals. The objective of this study was to see if monitoring the hearing status during intravenous infusion of EA could minimalize individual variability and to evaluate the correlation between the dose of EA and the body weight (wt). MATERIALS AND METHOD: Twenty cats with the mean age of 24 weeks+/-3.7 (range, 20.6-28.3 weeks) and the mean weight of 3.27 kg+/-0.75 (range 2.4-4.75 kg) received a subcutaneous injection of KM (300 mg/kg) followed by an intravenous infusion of EA (1 mg/min). Click evoked auditory brainstem responses (ABRs) were recorded to monitor the hearing during the infusion. When the ABR thresholds rose to levels in excess of 90 dB SPL, the infusion of EA was stopped. The histopathologies for sections of apex, middle, base of cochlea were examined after 6 months. RESULTS: There was a significant positive correlation (p<.001, r2=.583) between the EA dose and body weight. Cochlear histopathology showed an absence of organ of Corti and decrease of spiral ganglion cells in the majority of cochleas, especially in the basal turn. The extent of loss of spiral ganglion cells was dependent on their distance from the round window. CONCLUSION: Monitoring the animal's hearing status during the procedure ensured that the dose of EA was optimized for individual animals. Thus, the positive correlation between the EA dose and body weight should be considered should in designing the animal models of controlled high frequency hearing loss.

The Analysis of Prognostic Factor and Treatment Outcome of Malignancies of the External Auditory Canal / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The purpose of this study was to analyze the survival rate treatment modalities and certain prognostic factors in patients with external auditory canal (EAC) cancer using the University of Pittsburgh TNM Staging System. SUBJECTS AND METHOD: Medical records and radiological findings of 33 patients treated for EAC cancer between January 1995 and January 2009 were reviewed retrospectively. The median follow-up period was 65 months (range, 12-182 months). RESULTS: The most common histological type was squamous cell carcinoma (19 patients; 57.6%), followed by adenoid cystic carcinoma (9 patients; 27.3%), basal cell carcinoma (1 patient), adenocarcinoma (1 patient), rhabdomyosarcoma (1 patient), undifferentiated carcinoma (1 patient) and malignant small round cell tumor (1 patient). Seven of 33 patients died of EAC cancer and the overall survival rate was 70.6%. There was a significant difference in the survival rate with respect to TNM stages (p=.031). The 10 yr-survival rate of patients with stage I disease was 100.0%, whereas those of stages II, III and IV were 50.0, 80.0 and 58.3%, respectively. Five of 7 patients with recurrence had a history of initial positive resection margin. CONCLUSION: Early detection and proper surgical treatment with sufficient resection margin are essential for disease-specific survival and prevention of recurrence in patients with EAC cancer.

Analysis of P1 Latency in Normal Hearing and Profound Sensorineural Hearing Loss

OBJECTIVES: P1 is a robust positivity at a latency of 50-150 msec in the auditory evoked potential of young children. It has been reported that over the first 2-3 years of life, there is a rapid decrease of the latency and the mean P1 latency in adults with normal hearing is approximately 60 msec. This study was designed to evaluate the change of the P1 latency in Koreans with normal hearing according to age and to compare this with the P1 latency of young patients with profound sensorineural hearing loss before and/or after cochlear implantation. METHODS: Among the patients who visited the Department of Otorhinolaryngology at Seoul National University Hospital from June 2007 to September 2009, the P1 response was recorded in 53 patients in the normal hearing group, in 13 patients in the pre-cochlear implantation (CI) group and in 10 patients in the post-CI group. A synthesized consonant-vowel syllable /ba/ was used to elicit the evoked responses. The evoked responses were collected using the center of the frontal head. For each subject, an individual grand average waveform was computed by averaging the ten recordings. The P1 latency was visually identified as a robust positivity in the waveform. RESULTS: For the normal hearing group, the P1 latency showed the pattern of shortening as the age increased (coefficient, -0.758; P<0.001). For the pre-CI group, 10 cases showed delayed latencies and 3 cases did not show the P1 wave. For the post-CI group, the P1 latencies showed a less delayed tendency than those of the pre-CI group, but this was not statistically different. CONCLUSION: This report provides the standard value of the P1 latency at each age in Koreans for the first time and the findings support that the maturation of the central auditory pathways could be measured objectively using the P1 latency.

A Case of Cholesteatoma Extended Both into the Cochlea and IAC / 대한이비인후과학회지

Labyrinthine fistula caused by cholesteatoma can make various complications according to the site of the lesion. The lateral canal, superior canal ampulla, posterior canal and promontory of the cochlea are the common areas of fistulization. However, invasion into the cochlea or internal auditory canal (IAC) is very rare. We have experienced a case of cholesteatoma extending into the cochlea and IAC, which was successfully treated via the transotic approach. Herein we report this case with a review of literature