Case report: A fatal case of cryptococcosis in an immunocompetent patient due to Cryptococcus deuterogattii (AFLP6/VGII).

INTRODUCTION: Cryptococcosis in immunocompetent adults is a rare disease in Europe, mostly induced by members of the Cryptococcus gattii species complex. The diagnosis can be challenging due to its rarity, unspecific symptoms and long symptomless latency. CASE PRESENTATION: A 49-year-old woman with a three weeks history of headache was admitted to the hospital due to discrete ataxia and impaired vision. Cranial magnetic resonance imaging (MRI) showed a contrast-enhancing mass in the cerebellum. Further investigations detected a slight leukocytosis and a single subpleural nodule in the right inferior lung lobe. The cerebral lesion was surgically removed, and a direct frozen section only showed an unspecific inflammation. In the course of her admission she developed non-treatable cerebral edema and died ten days after surgical intervention. Histopathological examination of the surgical specimen and postmortem evaluation of the lung and the cerebrum demonstrated fungal elements. Molecular identification of the fungal elements in formalin-fixed paraffin-embedded tissue lead to the diagnosis of cryptococcosis induced by C. gattii sensu lato. Molecular genetic analysis identified the involved cryptococcal species as genotype AFLP6/VGII, recently described as Cryptococcus deuterogattii, which is known to be endemic to the west-coast of Canada and the USA. Additional heteroanamnestic information revealed that she had spent her holidays on Vancouver Island, Canada, two years before disease onset, indicating that infection during this stay seems to be plausible. CONCLUSION: Cryptococcosis due to C. deuterogattii is a rarely encountered fungal disease in Europe, not particularly associated with immunodeficiency, and infection is likely to be contracted in endemic areas. Due to its rarity, long symptomless latency, unspecific symptoms and misleading radiological features the diagnosis can be challenging. Physicians need to be aware of this differential diagnosis in immunocompetent patients, as early adequate therapy can be lifesaving.

Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.

Intracochlear schwannoma : Tumor removal via subtotal cochleoectomy and partial cochlear reconstruction with preservation of semicircular canal function.

We report about a patient, who in 2005, as a 37-year-old, experienced a sudden, mild to moderate, mid-frequency sensorineural hearing loss in the right ear, along with tinnitus and mild dizziness. MRI of the temporal bone revealed a very small (1 mm) enhancing lesion in the second turn of the right cochlea after injection of contrast medium. Hearing gradually deteriorated, with complete hearing loss in 2015. At this time, an MRI scan showed a lesion completely filling the cochlea.The tumor was removed through a subtotal cochleoectomy. The cochlea was partially reconstructed with cartilage and fascia. A cochlea implant dummy electrode was inserted as a placeholder in order to try to preserve a channel within the most likely fibrosing "neocochlea" during follow-up MRI examinations.Vestibular function tests 2 months postoperatively showed preserved caloric excitability and a normal vestibulo-ocular reflex (video head impulse test, vHIT) in all three planes. The patient is free of vertigo.Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss. Surgical removal of an intracochlear ILS through partial or subtotal cochleoectomy with preservation of vestibular function is possible.

[Intracochlear schwannoma : Tumor removal via subtotal cochleoectomy and partial cochlear reconstruction with preservation of semicircular canal function. German version]. / Intracochleäres Schwannom : Tumorentfernung über subtotale Cochleoektomie und partielle Rekonstruktion der cochleären Kapsel unter Erhalt der Bogengangsfunktion.

We report about a patient who in 2005, aged 37 years, experienced sudden mild to moderate, mid-frequency sensorineural hearing loss in the right ear along with tinnitus and mild dizziness. MRI of the temporal bone revealed a very small (1 mm) contrast-enhancing lesion in the second turn of the right cochlea. Hearing gradually deteriorated with complete hearing loss in 2015. At this time, an MRI scan showed a lesion completely filling the cochlea.The tumor was removed via transmeatal subtotal cochleoectomy. The cochlea was partially reconstructed with cartilage and fascia. A cochlea implant dummy electrode was inserted as a placeholder in order to prevent complete fibrosis of the "neocochlea" during follow-up MRI examinations.Vestibular function tests 2 months postoperatively showed preserved caloric excitability and a normal vestibulo-ocular reflex (vHIT) in all three planes. The patient is free of vertigo.Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss, which should be specifically checked for in MRI. Surgical removal of an intracochlear ILS through partial or subtotal cochleoectomy with preservation of vestibular function is possible in principle.

[Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants. German version]. / Intralabyrinthäre Schwannome : Chirurgisches Management und Hörrehabilitation mit Cochleaimplantaten.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebello-pontine angle (CPA), 1 a transotic including the CPA and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients opted for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single stage procedure; 1 patient received extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI following repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontile angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Provided performed early enough, cochlear implantation after surgical removal of ILS is an option for auditory rehabilitation, thus representing-in contrast to the "wait-and-test-and-scan" strategy-a therapeutic approach.