Giant cranionasal epithelioid haemangioendothelioma with invasive growth pattern mimicking a skull base chondrosarcoma.

Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that is composed of mostly epithelioid cells. EHE may arise as a solitary tumour or in the form of multiple body lesions, and commonly occurs in soft tissues, liver, pleura, lung, peritoneum, lymph nodes, breast, and many other sites. EHE in the cranionasal region is extremely rare. There are very few reports of cases of skull-base EHE. We discuss an extremely rare presentation of an aggressive EHE that originated from the sellar region. Based on literature review, our patient is the first reported case of a giant solitary EHE with prepontine cistern invasion and abducens nerve encroachment mimicking a chondrosarcoma. We treated this rare tumour by near subtotal surgical excision with subsequent radiotherapy, considering that complete tumour resection with free margins in both cavernous sinus and clival region avoiding neural and vascular structure encroachment becomes technically difficult.

Enlarged vestibular aqueduct as a cause of postneonatal deafness.

INTRODUCTION: The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5-15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located. OBJECTIVE: To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations. METHOD: Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023. RESULTS: Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation. DISCUSSION: The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.

Comparison of the MIPS technique versus linear incision with tissue preservation in osseointegrated implants in the Ponto model®.

BACKGROUND AND OBJECTIVES: The surgery of osseointegrated implants has undergone different modifications over the years with the aim of achieving better results and facilitating the surgical technique. Today the most commonly used technique is the linear incision with tissue preservation and placement of the abutment and implant. The long-term success of this technique has served as the basis for the development of the so-called minimally invasive surgical approach (MIPS). This study compares the short-, medium- and long-term results between the classic linear incision technique and the MIPS technique. MATERIAL AND METHODS: A prospective study was conducted on patients who had an osseointegrated implant placed between February 2016 and February 2020. A total of 59 surgeries were performed, 32 surgeries according to the linear incision technique with tissue preservation and 27 with MIPS technique. Outcomes were evaluated at one week, one month and one year. RESULTS: Statistically significant differences were achieved between the 2 groups at one week after surgery. Eighty per cent of the MIPS patients had Holgers grades 0-1 compared to 35% of the linear technique patients (p = 0.001). No statistically significant differences were observed at one month (p = 0.457) and one year (p = 0.228). One case with grade 4 was recorded which resulted in implant extrusion one month after surgery with the MIPS technique. A new osseointegrated implant was placed 2 months after the fall using the same MIPS technique with good results. We were also able to verify that the duration of surgery was much shorter with the MIPS technique and better tolerated in terms of postoperative discomfort by the patient. CONCLUSIONS: In our experience, the MIPS technique is the technique of choice for surgery of osseointegrated Ponto model implants as it is simpler, faster and presents fewer problems in the immediate postoperative period, with similar long-term postoperative results.

Universal neonatal hearing screening and delayed hearing loss or late-developmental hearing loss.

OBJECTIVE: To determine the percentage of children with permanent bilateral postnatal hearing loss in order to study its incidence, related risk factors, diagnosis and treatment. METHODS: Retrospective study to collect data on children diagnosed with hearing loss outside the neonatal period in the Hearing Loss Unit of the Hospital Universitario Central de Asturias, from April 2014 to April 2021. RESULTS: 52 cases met the inclusion criteria. The detection rate of congenital hearing loss in the neonatal screening programme in the same study period was 1.5 children per thousand newborns per year, adding postnatal hearing loss results in a rate of infant bilateral hearing loss of 2.7 children per thousand (55.5% and 44.4% respectively). Thirty-five children presented risk factors for hearing loss, of which 23 were at retrocochlear risk. The mean age at referral was 91.9 (18-185) months. Hearing aid fitting was indicated in 44 cases (84.6%). Cochlear implantation was indicated in eight cases (15.4%). DISCUSSION: Although congenital hearing loss accounts for the majority of childhood deafness, postnatal hearing loss has a significant incidence. This may be mainly due to: (1) that hearing impairment may arise in the first years of life, (2) that mild hearing loss as well as hearing loss in severe frequencies are undetectable by neonatal screening in some cases, (3) that some children may have false negative results. CONCLUSION: postnatal hearing loss requires identification of risk factors and long-term follow-up of children with hearing loss, as it needs to be detected and treated early.

Ototoxicity in cancer survivors: Experience and proposal of a surveillance protocol.

INTRODUCTION: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present our experience in ototoxicity monitoring. MATERIAL AND METHODS: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. RESULTS: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. CONCLUSION: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss.

Ludwig's angina as an extremely unusual complication for direct microlaryngoscopy.

BACKGROUND: An extremely rare case that to our knowledge has not been reported before is described, in which a patient had a Ludwig's angina as a complication of direct microlaryngoscopy. METHODS: We report a Ludwig's angina after a direct microlaryngoscopy for a Reinke's edema, due to erosion on the internal face of the mandible produced by compression of the laryngoscope. RESULTS: The patient underwent placement of 2 drainages, intraoral and cervical, and several incisions on the floor of the mouth, with intravenous corticosteroids and antibiotics and with resolution of the illness without performing tracheostomy. CONCLUSIONS: Ludwig's angina is an extremely rare complication of microlaryngoscopy, but it is potentially life-threatening. Early diagnosis and treatment resulted in survival of the patient without complications.

[Ototoxicity in cancer survivors: experience and proposal of a surveillance protocol]. / Ototoxicidad en pacientes oncológicos: experiencia y propuesta de un protocolo de vigilancia.

INTRODUCTION: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present experience in ototoxicity monitoring. MATERIAL AND METHODS: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. RESULTS: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. CONCLUSION: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss.

Application of Wireless Contralateral Routing of Signal (CROS) Technology in Unilateral Cochlear Implant Users. / Aplicación de la tecnología de enrutamiento inalámbrico contralateral de la señal (CROS) en usuarios de implante coclear unilateral.

INTRODUCTION: Single cochlear implantation usually provides substantial speech intelligibility benefits but bilaterally deaf, unilaterally implanted subjects will continue to experience limitations due to the head shadow effect, like single-sided deaf individuals. In the treatment of individuals with single-sided deafness one option is contralateral routing of signal (CROS) devices, which constitute a non-surgical intervention of the second ear in unilaterally implanted individuals. METHOD: Twelve experienced adult cochlear implant users with Naída Q70 processor and the CROS device used in combination participated in the study. For the study 3 conditions were provided: cochlear implant only, omnidirectional microphone mode (CROS deactivated); cochlear implant plus CROS activated, omnidirectional microphone mode and cochlear implant plus CROS activated, UltraZoom mode. Speech reception thresholds were determined in quiet and noise. Subjective feedback regarding the practical usability of the CROS device and the perceived benefit were collected. RESULTS: There was a 27.6% improvement in speech understanding in quiet and 32.5% improvement in noise when CROS device was activated. Using advanced directional microphones, a statistically significant benefit of 35% was obtained. The responses to the questionnaires revealed that the subjects perceived benefit in their everyday lives when using the CROS device with their cochlear implants. CONCLUSION: The investigated CROS device used by unilateral CI recipients in cases where bilateral implantation is not an option provides both subjective and objective speech recognition benefit when the signal is directed to the CROS device. Unfavourable conditions where speech is presented from the cochlear implant side and noise from the CROS side or diffusely were not included in this evaluation since the CROS device adds additional noise and performance is expected to decrease as has previously been shown.

Conservative treatment of retropharyngeal and parapharyngeal abscess in children.

Parapharyngeal and retropharyngeal infections, which can potentially cause life-threatening complications, could be treated conservatively with no need for surgical drainage. A retrospective analysis of all patients diagnosed with retropharyngeal and parapharyngeal infections was performed. Information regarding age, sex, presenting symptoms, physical examination, laboratory and imaging evaluations, management, duration of hospital stay, and complications were reviewed. Seven children were identified, 4 with retropharyngeal abscess and 3 with parapharyngeal abscess. All but 1 patient were under 7 years old, and all were treated with intravenous amoxicilin/clavulanic acid and corticosteroids. Torticollis and fever were present in all the patients. The mean length of hospital stay was 7 days. There were no complications associated. We demonstrate that retropharyngeal and parapharyngeal abscesses can be treated medically, reserving the surgical drainage for complicated cases. Treatment with intravenous antibiotics and corticosteroids is a safe option, reducing the durations of symptoms and the length of hospital stay.

Simultaneous pleomorphic adenomas of the hard palate and parapharyngeal space.

We report a very unusual presentation of simultaneous pleomorphic adenomas of 2 different locations, hard palate and parapharyngeal space. Patient age of presentation is rare because these tumors are seen in younger patients. We treated a 70-year-old woman with these 2 tumors, resecting both lesions with intraoral and cervical approaches. Pleomorphic adenomas are frequent lesions, but in the literature reviewed, we have not found articles reporting 2 simultaneous pleomorphic adenomas of these locations. Complete surgical resection is very important to avoid recurrences.

Malignant triton tumor of the infratemporal fossa.

Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells. It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis. Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival. However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor. The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors. We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.

[Primary tumours of the parapharyngeal space. Our experience in 51 patients]. / Tumores primarios del espacio parafaríngeo. Nuestra experiencia en 51 pacientes.

INTRODUCTION AND OBJECTIVES: Tumours of the parapharyngeal space are rare, representing only 0.5 % of head and neck oncology. The aim of this study is to present our experience with the diagnosis and therapies for these tumours. PATIENTS AND METHOD: This study is a retrospective review of 51 patients diagnosed as having tumours of the parapharyngeal space and treated surgically between 1984 and 2006. Tumours originating in the deep lobe of parotid gland were excluded. RESULTS: Seventy percent of the parapharyngeal space neoplasms were benign and thirty percent malignant. Pleomorphic adenoma was the most common neoplasm (37 %), followed by miscellaneous benign tumours (34 %), paraganglioma (21 %), and neurogenic tumours (8 %). Surgery was chosen in all case, with the transcervical approach used in 32 cases, the cervical-transparotid approach in 13 cases, the transmandibular approach in 4 cases (with mandibulectomy in 1 patient), the transoral approach in 1 case, and the infratemporal fossa approach in 1 case. The most common complications were those deriving from damage to nerve structures. CONCLUSIONS: Most parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.

[Surgical treatment of vagal paragangliomas]. / Tratamiento quirúrgico de los paragangliomas vagales.

Vagal paragangliomas are much less frequent than carotid and jugulo-tympanic paragangliomas and, like these other forms, can be sporadic or familial. The familial forms usually occur as isolated tumors or within syndromic entities such as multiple endocrine neoplasia type IIA-B, Von Hippel- Lindau syndrome and neurofibromatosis type 1. The genes causing the disease are located in three loci: SDHD (11q23), SDHC (1q21) and SDHB (1p36.1p35). Approximately one-third of apparently sporadic paragangliomas also show germline SDH mutations. In familial forms, the rate of multicentric tumors is up to 30-40%. Vagal paragangliomas arise from arise from paraganglia associated with the vagus nerve. Lower cranial nerve deficits are frequently observed. An essential feature in the diagnosis of vagal paraganglioma is that they are situated behind the internal carotid artery. The treatment of vagal paraganglioma should be based on the biological behavior of the tumor, the age and health status of the patient, tumoral size and the possible treatment-associated morbidity. Surgery is important in the treatment of vagal paraganglioma but this option is controversial in patients without lower cranial nerve palsy. The approach of choice is usually cervical or transcervical. If the tumor involves the jugular fossa, in addition to the cervical approach, a transpetrous approach is also required. Expectant management and radiotherapy are often valid options.

Validation of the measures of cepstral peak prominence as a measure of dysphonia severity in Spanish-speaking subjects. / Validez de las medidas del pico cepstral para la valoración objetiva de la disfonía en sujetos de habla hispana.

INTRODUCTION AND OBJECTIVES: Perceptual rating of overall voice quality and other more specific perceptual dimensions is difficult, as such judgments depend on the listener's subjectivity. Thus, finding objective, valid, and accessible clinical measures to include in comprehensive voice evaluation protocols is a priority. The purposes of this study were to 1) determine the diagnostic accuracy of a single acoustic measure, smoothed cepstral peak prominence (CPPS), to predict voice disorder status from sustained vowels and connected speech samples using the software Praat; 2) to determine the relationship between measures of CPPS and perceptual ratings of vocal quality; and 3) describe the normative values of CPPS. METHOD: Measures of CPPS were obtained from connected speech and sustained vowel recordings of 72 Spanish-speaking subjects with voice disorders and 52 nondysphonic Spanish-speaking subjects with no vocal disorders using freely downloadable Praat software. IBM SPSS Statistics software version 23 was used to complete the statistical analyses. RESULTS: results revealed a 70% sensitivity rate, a specificity rate of 85%. Estimated severity for sustained vowels and connected speech were strongly correlated and significantly associated with listener ratings of dysphonia severity. CONCLUSIONS: A single acoustic measure of CPPS was highly predictive of voice disorder status using Praat software. Clinicians may consider using CPPS to complement clinical voice evaluation and screening protocols.

Vowel production in hearing impaired children: A comparison between normal-hearing, hearing-aided and cochlear-implanted children. / Comparación de la producción vocal en niños con hipoacusia prelocutiva con adaptación audioprotésica e implante coclear con normooyentes.

INTRODUCTION AND OBJECTIVES: Inadequate auditory feedback in prelingually deaf children alters the articulation of consonants and vowels. The purpose of this investigation was to compare vowel production in Spanish-speaking deaf children with cochlear implantation, and with hearing-aids with normal-hearing children by means of acoustic analysis of formant frequencies and vowel space. METHODS: A total of 56 prelingually deaf children (25 with cochlear implants and 31 wearing hearing-aids) and 47 normal-hearing children participated. The first 2 formants (F1 and F2) of the five Spanish vowels were measured using Praat software. One-way analysis of variance (ANOVA) and post hoc Scheffé test were applied to analyze the differences between the 3 groups. The surface area of the vowel space was also calculated. RESULTS: The mean value of F1 in all vowels was not significantly different between the 3 groups. For vowels /i/, /o/ and /u/, the mean value of F2 was significantly different between the 2 groups of deaf children and their normal-hearing peers. CONCLUSION: Both prelingually hearing-impaired groups tended toward subtle deviations in the articulation of vowels that could be analyzed using an objective acoustic analysis programme.

Evaluation of vaccination coverage in cochlear implant patients at a referral hospital in Northern Spain. / Evaluación de las coberturas de vacunación en pacientes con implante coclear de un hospital de referencia del norte de España.

BACKGROUND AND OBJECTIVE: The risk of bacterial meningitis increases in cochlear implant patients. Therefore, pneumococcal, influenza and Haemophilus influenzae type b vaccination is indicated in this group. The aim of this study was to determine compliance with the vaccination calendar in patients implanted in a referral hospital. MATERIALS AND METHODS: Patients with cochlear implant operated between 2005 and 2015 were included. Vaccine coverage for seasonal influenza, Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes and pneumococcal polysaccharide 23-serotypes was evaluated. The sample was divided into 2 age groups (<14 years and≥14 years). A univariate and bivariate analysis was performed. RESULTS: Of the 153 patients studied (28.01% 0-13 years old and 71.9%≥14), only 2 (5.71%) had 100% adherence to the vaccination schedule, while 65.71% had compliance of 50% or less. Overall, vaccination coverage against the sequential pneumococcal pattern was 48.57%. The paediatric population exceeded 90% coverage for the vaccine against Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes while in those over 14 years of age it barely exceeded 50%. Influenza coverage was less than 40%. An inverse correlation was obtained between age and compliance, although not statistically significant. CONCLUSIONS: Vaccination coverage in patients with cochlear implant is lower than expected. Close collaboration between Otolaryngology departments and the Vaccination Units is proposed as the main strategy for improvement.

Radiation-induced sarcomas of the head and neck.

The aim of this study was to report our experience on the management of radiation-induced sarcomas (RISs). A retrospective study from 1994 to 2003 was done at our institution reviewing the medical records of 5 patients who had RISs. Five patients diagnosed of head and neck cancer received irradiation to treat their diseases. Later on, these patients developed new neoplasms in the irradiation fields (3 malignant fibrous histiocytoma, 1 osteosarcoma, and 1 angiosarcoma). The mean period of latency between irradiation and diagnosis of new tumors was 13 years. Early symptoms included neck or face swelling, odynophagia, or trigeminal hypoesthesia. All of the patients underwent surgical treatment. In 4 cases, regional and free flaps for head and neck reconstruction were required. Three patients also needed neoadjuvant chemotherapy. In the follow-up, 2 patients are alive and free of disease. Wide excision is the treatment of choice for RISs. Previous radiation therapy limits the dose that can be administered to the involved area, and the response rate to the chemotherapy is always poor.

[Usefulness of video-assisted cervicotomy on the extraction of foreign bodies]. / Utilidad de la cervicotomía videoasistida en la extracción de cuerpos extraños.

Foreign bodies located in the upper aerodigestive tract, if not diagnosed and taken out speedily, may be the cause of great morbimortality. We present a female patient who arrived at the emergency department reporting she had swallowed a fish bone; it was finally located in the pre-vertebral muscle at the level of C3-C4, where it had entered from the oesophagus at 2 cm from the superior oesophageal sphincter. We used a cervicotomy assisted by endoscopy, showing a new application for videoendoscopy.

[Sinonasal mucosal melanomas. Review of 17 case]. / Melanomas mucosos rinosinusales. Revisión de 17 casos.

INTRODUCTION: Mucosal melanomas (MM) represent 1.3 % of all melanomas; 55 % of them are located in the head and neck region mainly in the sinonasal and oropharyngeal cavity. Sinonasal mucosal melanomas have a high rate of local recurrence and a high risk of developing metastases, two reasons explaining the low survival rate. MATERIAL AND METHOD: We present 17 cases of sinonasal mucosal melanomas that were diagnosed and treated in our department from 1984 to July, 2008. The patients were studied by age, gender, site of presentation of the tumour, symptoms, radiology findings, treatment and course. RESULTS: Seventeen patients with sinonasal mucosal melanomas were studied. Of these 64.7 % were females and the mean age at presentation was 74 (range 48 to 93 years). Twelve cases developed in the nasal cavity and 5 in the paranasal sinuses. The most frequent site of presentation was the septum (35 %), and the most common symptom was epistaxis (77 %). Three patients rejected any treatment, 12 of the patients were treated surgically, one received chemotherapy and another chemotherapy plus radiotherapy. The 5 year disease-specific survival rate was 35.7 %. DISCUSSION AND CONCLUSIONS: Sinonasal mucosal melanomas are rare tumours with a high mortality. The treatment of choice is still surgical resection with wide surgical margins. Even so, the main cause of treatment failure is local recurrence, followed by the development of metastases. Because of the poor prognosis with this tumour, new treatment strategies are necessary.

[Reversible retrobulbar optic neuritis due to sphenoidal sinus disorders: two case studies]. / Neuritis óptica retrobulbar reversible por sinupatía esfenoidal: dos casos clínicos.

Optic neuritis are clinically demonstrated by a temporary but severe loss of vision and can be caused by a wide variety of diseases. It is unusual for sphenoidal sinusitis to co-exist with acute optic neuritis, so the simultaneous appearance of both diseases would invite aetiological suspicion. We present two cases where the first clinical manifestation of infectious sphenoidal pathology was retrobulbar optic neuritis, which reverted with treatment, medical in one case and surgical in the other, of the sinusitis.