Cortical Auditory Evoked Potential in Adults With Cochlear Implants: A Comparison With Adults With Normal Hearing

Background and Objectives@#To examine the maturational status of the auditory cortex in adults with cochlear implants (CIs) using the latencies of the P1, N1, and P2 components of cortical auditory-evoked potentials (CAEPs). @*Subjects and Methods@#A total of 25 adults with CIs and 25 age-matched, normal-hearing control subjects participated in this study. Specifically, patients with CIs were divided into three groups depending on their age of deafness onset: Group A comprised patients with prelingual deafness who had received CI during early childhood (n=7), Group B comprised patients with early childhood-onset, progressive deafness who had received CI during childhood (n=6), and Group C comprised patients with adult-onset deafness (n=12). The P1, N1, and P2 latencies of their CAEPs were then compared between CI patients and normal-hearing subjects. @*Results@#All participants showed clear CAEP responses. P1 and N1 latencies in Group A and Group C patients were significantly longer than those in the control group. Meanwhile, Group B patients had significantly longer N1 and P2 latencies, as compared with those in the control group. @*Conclusions@#Patients with prelingual deafness and those with early-childhood onset, progressive deafness who received CI developed primary and higher-order auditory areas postoperatively when they became adults. However, their auditory cortex maturational statuses seemed to be worse than that of normal-hearing individuals. Furthermore, adult patients with late-onset deafness might experience degenerative auditory cortex changes during the auditory deprivation period between deafness onset and cochlear implantation.

Outcomes of Anterior Scutum-Anchoring Tympanoplasty for Reconstruction of Tympanic Membrane Perforation Extending to Anterosuperior Quadrant / 대한이비인후과학회지

Background and Objectives@#It can be troublesome to repair tympanic membrane (TM) perforation extending to anterosuperior quadrant (ASQ). Underlay technique may cause re-perforation at ASQ, whereas overlay technique may result in anterior blunting or lateral healing of TM, causing conductive hearing loss. Anterior scutum-anchoring tympanoplasty (ASAT) can be a good alternative to repair TM perforation involving ASQ. ASAT is a variant of classic underlay tympanoplasty during which a graft is overlayed only at anterosuperior aspect by way of inserting a graft between the bony and fibrous annulus and anchoring the anterosuperior end of graft on the anterior scutum. This study was performed to assess the outcome of ASAT.Subjects and Method The medical records of patients with chronic otitis media who received type 1 tympanoplasty between April 2014 and August 2019 were reviewed. Depending on the size of the TM perforation, patients were classified into four groups. The tympanic graft success rate, complication rate, and hearing improvement were evaluated by group. @*Results@#The 82 ears of 78 patients were included for the study. At the most recent examination after 6 months of surgery, the graft success rate was 100% in all four groups. There were no postoperative complications. The mean hearing threshold was improved at 17.2 dB HL in air-conduction pure tone audiometry after surgery. @*Conclusion@#ASAT is a reliable and easily performable technique to repair TM perforation extending to ASQ with a high graft success rate and good postoperative hearing.

A Case of the Hamartoma of Middle Ear Presenting with the Aplasia of Ipsilateral Internal Carotid Artery / 대한이비인후과학회지

Hamartoma is a benign congenital tumor made of an abnormal mixture of normal tissues. It is a kind of developmental anomaly, in which the tissues are arranged with an excess growth of one or more of its components. Hamartoma is usually found in the lung, hypothalamus, and colon, but very rarely found in the head and neck areas. Symptoms are caused by the tumor tissue pressing against other tissues or organs. Surgical removal is the treatment modality of choice for hamartomas causing symptoms. The aplasia of unilateral internal carotid artery (ICA) is a rare congenital anomaly. ICA aplasia can cause structural change of the middle ear space. A 5 year-old female patient was presented with large hamartoma in the middle ear and the aplasia of the ipsilateral ICA. The mass was successfully removed without any complications. Authors report this case with a brief literature review.

A Case of Cochlear Implantation in a Child with Congenital Aural Atresia and Cochlear and Cochlear Nerve Malformation / 대한이비인후과학회지

Congenital aural atresia (CAA) is usually accompanied by an anomalous course of facial nerves, which may run over the round window niche toward the promontory in some cases of CAA. Very rarely, cochlear malformation might coexist at the same ear which has CAA, in which case cochlear implantation can be difficult due to anomalous facial nerve. Herein we present a case of cochlear implantation performed in a child who had CAA along with cochlear hypoplasia and cochlear nerve aplasia. The mastoid segment of facial nerve was displaced anteriorly, running over the round window niche. The surgery was done successfully using the round window approach without facial nerve injury.

A Case of Schwannoma of the Chorda Tympani Nerve / 대한이비인후과학회지

Schwannoma is a benign nerve sheath tumor composed of schwann cells. Most schwannoma arising in the middle ear are facial nerve schwannoma. In very rare occasions, schwannoma of the middle ear can arise from chorda tympani nerve. Hearing loss and tinnitus are the most common symptoms of patients with schwannoma of chorda tympani nerve and it can be treated by surgical excision. Recently, we treated a male patient with schwannoma of the chorda tympani nerve. This is the first case of schwannoma of the chorda tympani nerve reported in Korea. Herein, we present the case in detail with a review of the related literature.

The Korean Version of the Theory of Mind Test and the Early Development of the Theory of Mind in Children with Cochlear Implant / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Theory of Mind (ToM) refers to the ability humans have for recognizing the mental states of others and for predicting or explaining other people’s behavior. ToM is an essential ability people have for living with other people because it influences social relations, and the deaf children have been reported to have problems in ToM. As there are no ToM assessment tools in Korea, the purpose of this study was to establish such a version and to examine the early development of ToM of children with cochlear implant (CI). SUBJECTS AND METHOD: The original tools for ToM assessment were translated in Korean and the reliability and validity of the Korean version of ToM assessment tools were investigated with fifty normal hearing (NH) children. The early development of ToM of sixteen children with CI was compared with that of age-matched children with NH. RESULTS: The reliability of Korean version of ToM assessment tools was determined by tests for internal consistency and test-retest reliability. The validity of the tools was also evaluated by the tests for criterion-related validity and concurrent validity. There was no significant difference in ToM between children with CI and those with NH. CONCLUSION: The Korean version of ToM assessment tools was established and the assessment showed that the early development of ToM of deaf children, who received early intervention using CI, was comparable to that of NH children.

A Case of Squamous Cell Carcinoma of the Bilateral External Auditory Canals / 대한이비인후과학회지

A malignant tumor of the external auditory canal (EAC) is a rare tumor, with a prevalence of between 1 and 6 people per 1 million population. Common symptoms of the EAC cancer include otorrhea, hearing loss, otalgia, or tinnitus, which are similar to symptoms of otitis media or otitis externa. Therefore, diagnosis may be delayed due to lack of EAC cancer-specific symptoms and the prognosis is also relatively poor despite intensive treatment. The most malignant tumor of the EAC is squamous cell carcinoma (SCC), which usully invades the ear unilaterally. SCC of bilateral EAC is extremely rare. We report here a rare case of bilateral EAC SCC. In this case, unilateral EAC SCC was treated via stereotactic radiosurgery and contralateral EAC SCC developed subsequently. This case implicates that radiosurgery can be a treatment option for EAC SCC but it may have carcinogenic effect. This case also shows that the ear should be examined bilaterally even when unilateral EAC cancer is encountered.

A Case of Schwannoma of the Chorda Tympani Nerve / 대한이비인후과학회지

Schwannoma is a benign nerve sheath tumor composed of schwann cells. Most schwannoma arising in the middle ear are facial nerve schwannoma. In very rare occasions, schwannoma of the middle ear can arise from chorda tympani nerve. Hearing loss and tinnitus are the most common symptoms of patients with schwannoma of chorda tympani nerve and it can be treated by surgical excision. Recently, we treated a male patient with schwannoma of the chorda tympani nerve. This is the first case of schwannoma of the chorda tympani nerve reported in Korea. Herein, we present the case in detail with a review of the related literature.

The Korean Version of the Theory of Mind Test and the Early Development of the Theory of Mind in Children with Cochlear Implant / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES@#Theory of Mind (ToM) refers to the ability humans have for recognizing the mental states of others and for predicting or explaining other people’s behavior. ToM is an essential ability people have for living with other people because it influences social relations, and the deaf children have been reported to have problems in ToM. As there are no ToM assessment tools in Korea, the purpose of this study was to establish such a version and to examine the early development of ToM of children with cochlear implant (CI).SUBJECTS AND METHOD: The original tools for ToM assessment were translated in Korean and the reliability and validity of the Korean version of ToM assessment tools were investigated with fifty normal hearing (NH) children. The early development of ToM of sixteen children with CI was compared with that of age-matched children with NH.@*RESULTS@#The reliability of Korean version of ToM assessment tools was determined by tests for internal consistency and test-retest reliability. The validity of the tools was also evaluated by the tests for criterion-related validity and concurrent validity. There was no significant difference in ToM between children with CI and those with NH.@*CONCLUSION@#The Korean version of ToM assessment tools was established and the assessment showed that the early development of ToM of deaf children, who received early intervention using CI, was comparable to that of NH children.

Speech Perception and Language Outcome in Congenitally Deaf Children Receiving Cochlear Implants in the First Year of Life / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The objective of this study was to examine the effect of early cochlear implantation (CI) performed in infants less than 12 months of age. SUBJECTS AND METHOD: Twenty-five children who received their first CI before 12 months of age were included in this study (infant group). The speech perception and language outcomes of these children were compared with those of 14 children who received their first CI between 13 and 24 months of age (older group). All children received sequential bilateral CI with the inter-stage interval of less than 2 years. Speech perception was measured using Categories of Auditory Performance, monosyllabic word test and sentence test, and language ability was measured using Sequenced Language Scale for Infants, Preschool Receptive-Expressive Language Scale, or Receptive & Expressive Vocavulary Test, depending on the age at the time of testing. RESULTS: There were no significant differences in speech perception abilities between the infant group and the older group. The mean expressive language score of infant group was higher than that of the older group, but the difference was not statistically significant. However, the receptive language score of infant group was significantly higher than that of the older group. CONCLUSION: Children who received CI before 12 months of age achieved better receptive language ability than those who received it after 12 months of age. Thus CI should be performed as early as before 12 months of age to achieve better language ability.

Peer Relations in Children with Cochlear Implants / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Children who received cochlear implants (CIs) in early age can achieve age-appropriate language ability and can be educated in the classroom alongside normal hearing (NH) peers. However, what is rarely investigated is their relations with NH peers in the classroom. The purpose of this study was to examine the peer relations of children with CIs. SUBJECTS AND METHOD: Peer relations were examined using a peer relation scale test that included support, intimacy, recognition, conflict, and competition. Participants were 25 children who received their first CI before 3.5 years of age. Their peer relations were compared with those of 129 children with NH. RESULTS: Children with CIs evaluated themselves as having good peer relations, but their perception of peer relations varied according to gender and language ability. CI boys with language delay perceived lack of support and intimacy, whereas CI girls with language delay perceived more conflict than NH children. On the other hand, CI children with normal language ability showed no differences in their peer relations from NH children. CONCLUSION: Early CI surgery and intensive language rehabilitation can prevent peer problems and promote adjustment in school life for children with CI.

Interpersonal Competence in College Students with Cochlear Implants: A Preliminary Study / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: ObjectivesZZAlthough many studies have shown that cochlear implantation promotes restoration of adequate hearing and verbal ability, there is little research showing whether patients with cochlear implants (CI) adapted well to the society by forming a good interpersonal relationship. The purpose of this study was to investigate the interpersonal competence of college students who acquired good verbal ability through CI. SUBJECTS AND METHOD: Interpersonal competence was examined using the Korean version of interpersonal competence questionnaire. Participants were 23 college students with CI who had good verbal communication abilities. Their interpersonal competence was compared to that of 28 college students with normal hearing (NH). To identify the factors contributing to the interpersonal competence, regression analysis was performed using speech perception, vocabulary and speech intelligibility as independent variables. RESULTS: College students with CI showed significantly weaker interpersonal competence than those with NH. Among 5 sub-factors of interpersonal competence, assertion and conflict management were not significantly different between two groups and initiation, caring others, and appropriate disclosure of CI group were significantly weaker than NH group. But college students with CI who had normal vocabulary and Speech Intelligibility Ratings score of 5 showed good interpersonal competence, which was comparable to those with NH. CONCLUSION: College students with CI who acquired good verbal communication ability demonstrates lower interpersonal competence than those with NH. Therefore, it is necessary to support them to improve interpersonal competence as well as speech-language ability.

A Case of Auditory Neuropathy Caused by Pontine Hemorrhage in an Adult

A pontine hemorrhage can evoke several neurological symptoms because the pons contains various nuclei and nerve fibers. Hearing loss can develop as a result of a pontine hemorrhage because there is an auditory conduction pathway in the cochlear nucleus of the pons. However, very few cases of hearing loss caused by pontine lesions have been reported, and there have been no reports of auditory neuropathy that developed following a pontine hemorrhage. Recently we had a patient who experienced a nontraumatic pontine hemorrhage who was diagnosed with auditory neuropathy. The 34-year-old male patient was admitted to the emergency department with sudden alteration of mental status. His brain computed tomographic imaging revealed a hemorrhage in the central pons. He complained of hearing difficulties after his mental status recovered through conservative treatment, but a pure-tone audiogram showed very mild hearing loss in both ears. Further hearing tests using otoacoustic emissions, which showed normal responses, and auditory brainstem responses, which showed no waveforms at maximum stimulus intensity, revealed that his hearing difficulties were caused by auditory neuropathy. This case implies that the threshold of sound detection can be preserved in patients with pontine hemorrhage who complain of hearing difficulties. Auditory neuropathy should be considered as a possible cause of hearing difficulties in these patients and appropriate hearing tests should be performed.

Two Cases of Traumatic Stapedial Dislocation Caused by a Cotton Tipped Swab / 대한이비인후과학회지

A stapedial dislocation is a rare complication of ossicular trauma that is most commonly caused by direct, penetrating injury to the external ear canal. In this type of ossicular dislocation, patients usually suffer from cochleovestibular symptoms including progressive sensorineural hearing loss, conductive hearing loss, tinnitus, and vertigo. We present rare cases of internal stapedial dislocation after penetrating trauma, which resulted in the preservation of bone conduction hearing thresholds and complete resolution of the vestibular symptoms after an urgent repair. We have also reviewed the literature to determine the optimal management of stapedial dislocations.

Cochlear Implantation Using the Endomeatal Approach in a Child with a Extremely Hypoplastic Mastoid / 대한이비인후과학회지

The classic surgical technique for cochlear implantation was first introduced by House in 1961. This technique includes a mastoidectomy and a posterior tympanotomy, which has been most widely used for cochlear implantation. However, this approach may result in the injury of the chorda tympani nerve or the facial nerve. To avoid the risk of the neural injury, several alternative surgical methods have been developed. The endomeatal approach is one of the several alternative techniques. We present, along with a review of literatures, a case of cochlear implantation using the endomeatal approach performed in a child with an extremely hypoplastic mastoid.

Management of Pediatric Unilateral Sensorineural Hearing Loss / 대한이비인후과학회지

Every one of a thousand of neonates is born with unilateral sensorineural hearing loss (UHL) and the prevalence of UHL increases in school aged children due to delayed-onset or acquired HL. Some studies showed that UHL impacted on speech-language development and school performance adversely. Therefore, appropriate intervention for UHL children may be needed. Treatment approach for UHL children can be chosen depending on the degree of HL. Cochlear implantation (CI) provides benefits of binaural hearing including better speech perception in noise and sound localization to children with acquired severe-to-profound UHL. However, children with congenital severe-to-profound UHL and long duration of HL gain the unfavorable benefit from CI. Contralateral routing of signal hearing aid and bone anchored hearing device should not be recommended for young children with UHL because these devices can introduce noise to normal hearing ear and young children are not able to competently monitor their listening environment and to make judgments about when these devices may be appropriate. Conventional hearing aid (HA) has the potential of providing binaural hearing for UHL children with amplifiable hearing and the studies realized the binaural hearing when UHL children wore HA. However adherence to HA is poor because of some reasons including parents' reluctance to fit a HA in the presence of a normal-hearing ear and stigma effect. Long term follow up data of these interventions for UHL children are lacking. So fully informed consent is required during decision making process for UHL children until the concrete guideline of intervention for UHL children is established.

The Clinical Effectiveness of the Minimally Invasive Transcanal Myringotomy for the Removal of Early Stage Congenital Cholesteatoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Because of the wide application of the endoscopic diagnostic system and increased amount of attention for early stage congenital cholesteatoma (CC) recently, the detection of CC in stages I and II has increased. In this study, we investigated the effectiveness of minimally invasive transcanal myringotomy (MITM), and compared its results with those of postauricular approach (PAA) in early stage CC consisting of stage I and II of Potsic's classification. SUBJECTS AND METHOD: We retrospectively studied 70 patients who were diagnosed with CC after surgery performed by an otologist from June 2006 to June 2013. Thirty-two patients were in early stage CC consisting stage I and II. Of the 32 patients, MITM was performed on 20 and PAA was performed on 12. We analyzed the characteristics of disease, operation time, hospitalization period, recurrence and complications according to the stage and each operation. RESULTS: There were no significant differences between the stages and types of the disease between the MITM and the PAA group (p>0.05). The operation time and hospitalization period for the MITM group were significantly shorter than for the PAA group (p<0.001). There was no recurrence in the two groups during the average follow-up period of 33 months follow-up. Tympanic membrane perforation occurred in one patient in the MITM group. One patient presenting an external aural epidermal cyst in the PAA group was treated with surgical removal. CONCLUSION: The MITM is an efficacious and feasible method to remove early stage CC.

A Case of Nasopharyngeal Carcinoma Presenting as Middle Ear Tumor / 대한이비인후과학회지

Nasopharyngeal carcinoma (NPC) evolves most commonly from the pharyngeal recess posteromedial to the medial crura of the Eustachian tube opening in the nasopharynx. Therefore unilateral middle ear effusion with hearing loss is a common presenting symptom of NPC, and its presence in adult patients make physicians suspect NPC. Recently, we experienced a 72- year-old female patient with advanced NPC which invaded Eustachian tube and middle ear cavity. She had long standing chronic otitis media with large perforation of tympanic membrane. Because of tympanic membrane perforation, she did not have any Eustachian tube-related symptoms such as newly developing ear fullness or hearing loss and even the physician had overlooked the diagnosis of NPC. Her NPC was diagnosed at a very advanced stage after performing biopsy of middle ear tumor. This case report shows that NPC can present with very unusual findings like middle ear tumor.

A Case of Temporomandibular Joint Disorder Accompanied with Ear Bleeding / 대한이비인후과학회지

Temporomandibular joint disorder (TMD) is a generic term used for any problem concerning the jaw joint. The clinical signs of TMD are crepitus associated with movement, restriction of jaw movements, and pain within the joint cavity. But ear bleeding and an external auditory canal (EAC) mass as presenting symptoms are rare. We report the case of 78-year-old man with TMD, whose initial symptoms were ear bleeding, and temporomandibular pain intensifying when he is chewing, accompanied by an EAC mass. Physical examination revealed a reddish mass that originated from the anterior wall of EAC and bled when touched. Computed tomography showed a mass with soft tissue density and a bony defect in the anterior EAC wall. After conducting arthrocentesis and lavage repetitively, the wound was healed completely. It is likely that the patient's ear bleeding was due to patent foramen of Huschke or erosion of the anterior wall of EAC due to temporomandibular joint inflammation.

Expression Profile of Fas-Fas Ligand in Spiral Ganglion Cells During Apoptosis

OBJECTIVES: To examine the expression profile of Fas-Fas ligand (FasL) during glutamate (Glu)-induced spiral ganglion cell (SGC) apoptosis. METHODS: Cultured SGCs were treated with 10-mM, 25-mM, and 50-mM concentrations of Glu and incubated for 24 or 48 hours. The expression intensity of FasL, Fas, caspase 3, and morphology of single SGC were evaluated using immunofluorescence staining. RESULTS: In semiquantitative analysis of the Glu-treated SGC, FasL, and caspase 3 expression intensity were increased with concentration- and time-dependent manner. Fas expression intensity did not change with different concentration at 48 hours. In morphologic analysis of the Glu-treated SGC, number of apoptotic cells were increased with concentration- and time-dependent manner. CONCLUSION: FasL was expressed in apoptotic SGCs, suggesting that the Fas-FasL signaling pathway may be involved in the Glu-induced apoptosis of dissociated SGCs.